{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T17:31:05Z","timestamp":1793035865466,"version":"4.5.0"},"reference-count":241,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2016,10,1]],"date-time":"2016-10-01T00:00:00Z","timestamp":1475280000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"name":"Toray Science Foundation"},{"DOI":"10.13039\/100012207","name":"Grace Science Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100012207","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Grant-in-Aids for Scientific Research","award":["26110725"],"award-info":[{"award-number":["26110725"]}]},{"name":"Rakuten Inc., Tokyo, Japan"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Aspects of Medicine"],"published-print":{"date-parts":[[2016,10]]},"DOI":"10.1016\/j.mam.2016.05.004","type":"journal-article","created":{"date-parts":[[2016,5,27]],"date-time":"2016-05-27T22:06:19Z","timestamp":1464386779000},"page":"89-103","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":53,"special_numbering":"C","title":["Catabolism of N-glycoproteins in mammalian cells: Molecular mechanisms and genetic disorders related to the processes"],"prefix":"10.1016","volume":"51","author":[{"given":"Tadashi","family":"Suzuki","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"Pt 3","key":"10.1016\/j.mam.2016.05.004_bib0010","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1042\/bj2770743","article-title":"The substrate-specificity of human lysosomal alpha-D-mannosidase in relation to genetic alpha-mannosidosis","volume":"277","author":"al Daher","year":"1991","journal-title":"Biochem. J."},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0015","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1042\/bj2150573","article-title":"The catabolism of mammalian glycoproteins. Comparison of the storage products in bovine, feline and human mannosidosis","volume":"215","author":"Abraham","year":"1983","journal-title":"Biochem. J."},{"issue":"9","key":"10.1016\/j.mam.2016.05.004_bib0020","doi-asserted-by":"crossref","first-page":"5847","DOI":"10.1016\/S0021-9258(18)89099-8","article-title":"Degradation of short and long lived proteins in isolated rat liver lysosomes. Effects of pH, temperature, and proteolytic inhibitors","volume":"260","author":"Ahlberg","year":"1985","journal-title":"J. Biol. Chem"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib0025","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1111\/j.1469-8749.2004.tb00449.x","article-title":"Neurocognitive profiles in Salla disease","volume":"46","author":"Alajoki","year":"2004","journal-title":"Dev. Med. Child Neurol"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0030","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1093\/hmg\/7.1.75","article-title":"Human beta-mannosidase cDNA characterization and first identification of a mutation associated with human beta-mannosidosis","volume":"7","author":"Alkhayat","year":"1998","journal-title":"Hum. Mol. Genet"},{"issue":"14","key":"10.1016\/j.mam.2016.05.004_bib0035","doi-asserted-by":"crossref","first-page":"2615","DOI":"10.1096\/fasebj.3.14.2531691","article-title":"Lysosomal degradation of Asn-linked glycoproteins","volume":"3","author":"Aronson","year":"1989","journal-title":"FASEB J."},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0040","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1016\/0003-9861(89)90222-1","article-title":"Rat-liver chitobiase \u2013 purification, properties, and role in the lysosomal degradation of Asn-linked glycoproteins","volume":"272","author":"Aronson","year":"1989","journal-title":"Arch. Biochem. Biophys"},{"issue":"2\u20133","key":"10.1016\/j.mam.2016.05.004_bib0045","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/S0925-4439(99)00076-9","article-title":"Aspartylglycosaminuria: biochemistry and molecular biology","volume":"1455","author":"Aronson","year":"1999","journal-title":"Biochim. Biophys. Acta"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0050","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1111\/j.1651-2227.2002.tb01707.x","article-title":"Progressive nature of aspartylglucosaminuria","volume":"91","author":"Arvio","year":"2002","journal-title":"Acta Paediatr"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0055","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1001\/archneur.1979.00500380058006","article-title":"\u201cSalla disease\u201d: a new lysosomal storage disorder","volume":"36","author":"Aula","year":"1979","journal-title":"Arch. Neurol"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0060","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1042\/bj2540861","article-title":"Canine alpha-L-fucosidase in relation to the enzymic defect and storage products in canine fucosidosis","volume":"254","author":"Barker","year":"1988","journal-title":"Biochem. J."},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0065","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1111\/j.1432-1033.1986.tb09879.x","article-title":"Catabolism of glycoprotein glycans. Characterization of a lysosomal endo-N-acetyl-beta-D-glucosaminidase specific for glycans with a terminal chitobiose residue","volume":"159","author":"Baussant","year":"1986","journal-title":"Eur. J. Biochem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0070","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1016\/0014-5793(77)80729-1","article-title":"Activity of N-acetyl-beta-D-hexosaminidases A and B from human liver towards various oligosaccharides and a glycopeptide core derived from glycoproteins","volume":"84","author":"Bearpark","year":"1977","journal-title":"FEBS Lett"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0075","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1086\/302183","article-title":"Spectrum of mutations in alpha-mannosidosis","volume":"64","author":"Berg","year":"1999","journal-title":"Am. J. Hum. Genet"},{"issue":"11","key":"10.1016\/j.mam.2016.05.004_bib0080","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1016\/0020-711X(86)90255-7","article-title":"beta-Mannosidase in human serum and urine. A comparative study","volume":"18","author":"Bernard","year":"1986","journal-title":"Int. J. Biochem"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0085","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1093\/glycob\/cwq169","article-title":"Overexpression of Man2C1 leads to protein underglycosylation and upregulation of endoplasmic reticulum-associated degradation pathway","volume":"21","author":"Bernon","year":"2011","journal-title":"Glycobiology"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0090","first-page":"628","article-title":"Hexosaminidase isozyme in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease)","volume":"27","author":"Beutler","year":"1975","journal-title":"Am. J. Hum. Genet"},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib0095","doi-asserted-by":"crossref","first-page":"4758","DOI":"10.1016\/S0021-9258(17)38566-6","article-title":"The soluble form of rat liver alpha-mannosidase is immunologically related to the endoplasmic reticulum membrane alpha-mannosidase","volume":"261","author":"Bischoff","year":"1986","journal-title":"J. Biol. Chem"},{"issue":"28","key":"10.1016\/j.mam.2016.05.004_bib0100","doi-asserted-by":"crossref","first-page":"17110","DOI":"10.1016\/S0021-9258(17)44876-9","article-title":"Isolation, characterization, and expression of cDNA encoding a rat liver endoplasmic reticulum alpha-mannosidase","volume":"265","author":"Bischoff","year":"1990","journal-title":"J. Biol. Chem"},{"issue":"7","key":"10.1016\/j.mam.2016.05.004_bib0105","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1083\/jcb.201501101","article-title":"A little sugar goes a long way: the cell biology of O-GlcNAc","volume":"208","author":"Bond","year":"2015","journal-title":"J. Cell Biol"},{"issue":"24","key":"10.1016\/j.mam.2016.05.004_bib0110","doi-asserted-by":"crossref","first-page":"3156","DOI":"10.1101\/gad.10.24.3156","article-title":"Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis","volume":"10","author":"Bonten","year":"1996","journal-title":"Genes Dev"},{"issue":"11","key":"10.1016\/j.mam.2016.05.004_bib0115","doi-asserted-by":"crossref","first-page":"2017","DOI":"10.1007\/s00018-013-1538-3","article-title":"Lysosomal multienzyme complex: pros and cons of working together","volume":"71","author":"Bonten","year":"2014","journal-title":"Cell. Mol. Life Sci"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0120","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1038\/ejhg.2015.186","article-title":"Novel genetic causes for cerebral visual impairment","volume":"24","author":"Bosch","year":"2016","journal-title":"Eur. J. Hum. Genet"},{"issue":"6555","key":"10.1016\/j.mam.2016.05.004_bib0125","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1038\/378416a0","article-title":"A protein catalytic framework with an N-terminal nucleophile is capable of self-activation","volume":"378","author":"Brannigan","year":"1995","journal-title":"Nature"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0130","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1016\/j.bbamcr.2008.10.016","article-title":"Sorting of lysosomal proteins","volume":"1793","author":"Braulke","year":"2009","journal-title":"Biochim. Biophys. Acta"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0135","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1016\/S0006-291X(05)80060-9","article-title":"Bovine beta-mannosidase deficiency","volume":"173","author":"Bryan","year":"1990","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"7","key":"10.1016\/j.mam.2016.05.004_bib0140","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1016\/j.bbadis.2011.03.018","article-title":"GM1 gangliosidosis and Morquio B disease: an update on genetic alterations and clinical findings","volume":"1812","author":"Caciotti","year":"2011","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.mam.2016.05.004_bib0145","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1186\/1750-1172-8-114","article-title":"Galactosialidosis: review and analysis of CTSA gene mutations","volume":"8","author":"Caciotti","year":"2013","journal-title":"Orphanet J. Rare Dis"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0150","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.ejmg.2014.08.008","article-title":"NGLY1 mutation causes neuromotor impairment, intellectual disability, and neuropathy","volume":"58","author":"Caglayan","year":"2015","journal-title":"Eur. J. Med. Genet"},{"issue":"22","key":"10.1016\/j.mam.2016.05.004_bib0155","doi-asserted-by":"crossref","first-page":"8894","DOI":"10.1073\/pnas.0900288106","article-title":"Identification of protein O-GlcNAcylation sites using electron transfer dissociation mass spectrometry on native peptides","volume":"106","author":"Chalkley","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"7","key":"10.1016\/j.mam.2016.05.004_bib0160","doi-asserted-by":"crossref","first-page":"e11734","DOI":"10.1371\/journal.pone.0011734","article-title":"Identification of roles for peptide: N-glycanase and endo-beta-N-acetylglucosaminidase (Engase1p) during protein N-glycosylation in human HepG2 cells","volume":"5","author":"Chantret","year":"2010","journal-title":"PLoS ONE"},{"issue":"8","key":"10.1016\/j.mam.2016.05.004_bib0165","doi-asserted-by":"crossref","first-page":"3841","DOI":"10.1074\/jbc.270.8.3841","article-title":"Molecular cloning and characterization of bovine beta-mannosidase","volume":"270","author":"Chen","year":"1995","journal-title":"J. Biol. Chem"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib0170","doi-asserted-by":"crossref","first-page":"1837","DOI":"10.1515\/bchm2.1979.360.2.1837","article-title":"Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A","volume":"360","author":"Conzelmann","year":"1979","journal-title":"Hoppe Seylers Z. Physiol. Chem"},{"key":"10.1016\/j.mam.2016.05.004_bib0175","first-page":"89","article-title":"Glycolipid and glycoprotein degradation","volume":"60","author":"Conzelmann","year":"1987","journal-title":"Adv. Enzymol. Relat. Areas Mol. Biol"},{"issue":"19","key":"10.1016\/j.mam.2016.05.004_bib0180","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1056\/NEJM198611063151918","article-title":"Human beta-mannosidase deficiency","volume":"315","author":"Cooper","year":"1986","journal-title":"N. Engl. J. Med"},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib0185","doi-asserted-by":"crossref","first-page":"1580","DOI":"10.1016\/j.bbagen.2006.06.011","article-title":"Cloning and expression of mouse cytosolic alpha-mannosidase (Man2c1)","volume":"1760","author":"Costanzi","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib0190","doi-asserted-by":"crossref","first-page":"1455","DOI":"10.1093\/hmg\/11.12.1455","article-title":"Systemic and neurologic abnormalities distinguish the lysosomal disorders sialidosis and galactosialidosis in mice","volume":"11","author":"de Geest","year":"2002","journal-title":"Hum. Mol. Genet"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0195","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1042\/bj2210601","article-title":"Swainsonine-induced oligosaccharide excretion in sheep. Time-dependent changes in the oligosaccharide profile","volume":"221","author":"Daniel","year":"1984","journal-title":"Biochem. J."},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0200","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1093\/glycob\/2.4.327","article-title":"A human lysosomal alpha(1\u21926)-mannosidase active on the branched trimannosyl core of complex glycans","volume":"2","author":"Daniel","year":"1992","journal-title":"Glycobiology"},{"issue":"7","key":"10.1016\/j.mam.2016.05.004_bib0205","doi-asserted-by":"crossref","first-page":"3369","DOI":"10.1016\/S0021-9258(18)34783-5","article-title":"Evidence for two distinct forms of mammalian beta-mannosidase","volume":"257","author":"Dawson","year":"1982","journal-title":"J. Biol. Chem"},{"issue":"15","key":"10.1016\/j.mam.2016.05.004_bib0210","doi-asserted-by":"crossref","first-page":"4535","DOI":"10.1073\/pnas.79.15.4535","article-title":"Molecular defect in combined beta-galactosidase and neuraminidase deficiency in man","volume":"79","author":"D'Azzo","year":"1982","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"14","key":"10.1016\/j.mam.2016.05.004_bib0215","doi-asserted-by":"crossref","first-page":"9706","DOI":"10.1016\/S0021-9258(19)50148-X","article-title":"A human lysosomal alpha-mannosidase specific for the core of complex glycans","volume":"267","author":"De Gasperi","year":"1992","journal-title":"J. Biol. Chem"},{"issue":"16","key":"10.1016\/j.mam.2016.05.004_bib0220","doi-asserted-by":"crossref","first-page":"9329","DOI":"10.1016\/S0021-9258(18)60535-6","article-title":"Presence of two endo-beta-N-acetylglucosaminidases in human kidney","volume":"264","author":"DeGasperi","year":"1989","journal-title":"J. Biol. Chem"},{"issue":"14","key":"10.1016\/j.mam.2016.05.004_bib0225","doi-asserted-by":"crossref","first-page":"3729","DOI":"10.1111\/j.1742-4658.2009.07089.x","article-title":"Lysosomal localization of GLUT8 in the testis\u2013the EXXXLL motif of GLUT8 is sufficient for its intracellular sorting via AP1- and AP2-mediated interaction","volume":"276","author":"Diril","year":"2009","journal-title":"FEBS J."},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0230","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/0014-5793(79)80714-0","article-title":"The effects of ions and pH on the transport of sugars into rat liver lysosomes","volume":"106","author":"Docherty","year":"1979","journal-title":"FEBS Lett"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0235","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1042\/bj1780361","article-title":"The permeability of rat liver lysosomes to sugars. Evidence for carrier-mediated facilitated diffusion","volume":"178","author":"Docherty","year":"1979","journal-title":"Biochem. J."},{"issue":"Suppl. 2","key":"10.1016\/j.mam.2016.05.004_bib0240","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1007\/BF01804251","article-title":"Beta-mannosidosis in two brothers with hearing loss","volume":"11","author":"Dorland","year":"1988","journal-title":"J. Inherit. Metab. Dis"},{"issue":"7476","key":"10.1016\/j.mam.2016.05.004_bib0245","doi-asserted-by":"crossref","first-page":"1313","DOI":"10.1016\/S0140-6736(66)91718-1","article-title":"A new mucopolysaccharide lipid-storage disease","volume":"2","author":"Durand","year":"1966","journal-title":"Lancet"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0250","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1016\/S0022-3476(69)80464-6","article-title":"Fucosidosis","volume":"75","author":"Durand","year":"1969","journal-title":"J. Pediatr"},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib0255","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1038\/gim.2014.22","article-title":"Mutations in NGLY1 cause an inherited disorder of the endoplasmic reticulum-associated degradation pathway","volume":"16","author":"Enns","year":"2014","journal-title":"Genet. Med"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0260","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1111\/j.1432-1033.1992.tb17227.x","article-title":"Degradation of gangliosides by the lysosomal sialidase requires an activator protein","volume":"208","author":"Fingerhut","year":"1992","journal-title":"Eur. J. Biochem"},{"issue":"18","key":"10.1016\/j.mam.2016.05.004_bib0265","doi-asserted-by":"crossref","first-page":"12105","DOI":"10.1016\/S0021-9258(18)99071-X","article-title":"Characterization of the mutation responsible for aspartylglucosaminuria in three Finnish patients. Amino acid substitution Cys163\u2192Ser abolishes the activity of lysosomal glycosylasparaginase and its conversion into subunits","volume":"266","author":"Fisher","year":"1991","journal-title":"J. Biol. Chem"},{"issue":"27","key":"10.1016\/j.mam.2016.05.004_bib0270","doi-asserted-by":"crossref","first-page":"19607","DOI":"10.1016\/S0021-9258(18)41818-2","article-title":"Cloning and expression of the cDNA sequence encoding the lysosomal glycosidase di-N-acetylchitobiase","volume":"267","author":"Fisher","year":"1992","journal-title":"J. Biol. Chem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0275","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1016\/0014-5793(90)81211-6","article-title":"Cloning and sequence analysis of a cDNA for human glycosylasparaginase. A single gene encodes the subunits of this lysosomal amidase","volume":"269","author":"Fisher","year":"1990","journal-title":"FEBS Lett"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0280","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.bbagen.2008.09.009","article-title":"Glycobiology in the cytosol: the bitter side of a sweet world","volume":"1790","author":"Funakoshi","year":"2009","journal-title":"Biochim. Biophys. Acta"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0285","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/0005-2760(92)90210-M","article-title":"Activator proteins and topology of lysosomal sphingolipid catabolism","volume":"1126","author":"Furst","year":"1992","journal-title":"Biochim. Biophys. Acta"},{"issue":"2\u20133","key":"10.1016\/j.mam.2016.05.004_bib0290","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/0925-4439(94)00075-2","article-title":"Lysosomal storage diseases","volume":"1270","author":"Gieselmann","year":"1995","journal-title":"Biochim. Biophys. Acta"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0295","first-page":"250","article-title":"Lysosomal storage diseases","volume":"53","author":"Glew","year":"1985","journal-title":"Lab. Invest"},{"key":"10.1016\/j.mam.2016.05.004_bib0300","series-title":"The Metabolic and Molecular Bases of Inherited Diseases","first-page":"3827","article-title":"The GM2 gangliosidoses","author":"Gravel","year":"2001"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0305","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1093\/glycob\/1.5.487","article-title":"Cytosolic glycosidases: do they exist?","volume":"1","author":"Haeuw","year":"1991","journal-title":"Glycobiology"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0310","doi-asserted-by":"crossref","first-page":"1257","DOI":"10.1111\/j.1432-1033.1991.tb16498.x","article-title":"Substrate-specificity of rat-liver cytosolic alpha-D-mannosidase \u2013 novel degradative pathway for oligomannosidic type glycans","volume":"202","author":"Haeuw","year":"1991","journal-title":"Eur. J. Biochem"},{"issue":"Pt 3","key":"10.1016\/j.mam.2016.05.004_bib0315","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1042\/bj2970463","article-title":"The core-specific lysosomal alpha(1\u20136)-mannosidase activity depends on aspartamidohydrolase activity","volume":"297","author":"Haeuw","year":"1994","journal-title":"Biochem. J."},{"issue":"13","key":"10.1016\/j.mam.2016.05.004_bib0320","doi-asserted-by":"crossref","first-page":"2509","DOI":"10.1007\/s00018-015-1881-7","article-title":"Generation and degradation of free asparagine-linked glycans","volume":"72","author":"Harada","year":"2015","journal-title":"Cell. Mol. Life Sci"},{"issue":"11","key":"10.1016\/j.mam.2016.05.004_bib0325","doi-asserted-by":"crossref","first-page":"1196","DOI":"10.1093\/glycob\/cwv055","article-title":"Cytosolic-free oligosaccharides are predominantly generated by the degradation of dolichol-linked oligosaccharides in mammalian cells","volume":"25","author":"Harada","year":"2015","journal-title":"Glycobiology"},{"issue":"15","key":"10.1016\/j.mam.2016.05.004_bib0330","doi-asserted-by":"crossref","first-page":"8048","DOI":"10.1074\/jbc.M115.685313","article-title":"Non-lysosomal degradation of singly phosphorylated oligosaccharides initiated by the action of a cytosolic endo-beta-N-acetylglucosaminidase","volume":"291","author":"Harada","year":"2016","journal-title":"J. Biol. Chem"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0335","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1007\/BF00731639","article-title":"Different oligosaccharides accumulate in the brain and urine of a cat with alpha-mannosidosis: structure determination of five brain-derived and seventeen urinary oligosaccharides","volume":"8","author":"Hard","year":"1991","journal-title":"Glycoconj. J."},{"key":"10.1016\/j.mam.2016.05.004_bib0340","doi-asserted-by":"crossref","first-page":"183","DOI":"10.3389\/fendo.2014.00183","article-title":"Three decades of research on O-GlcNAcylation \u2013 a major nutrient sensor that regulates signaling, transcription and cellular metabolism","volume":"5","author":"Hart","year":"2014","journal-title":"Front. Endocrinol. (Lausanne)"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0345","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1007\/BF00691760","article-title":"Neurovisceral storage and dysmyelinogenesis in neonatal goats","volume":"25","author":"Hartley","year":"1973","journal-title":"Acta Neuropathol"},{"key":"10.1016\/j.mam.2016.05.004_bib0350","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1038\/ncomms1309","article-title":"alpha-Mannosidase 2C1 attenuates PTEN function in prostate cancer cells","volume":"2","author":"He","year":"2011","journal-title":"Nat. Commun"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0355","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1002\/ajmg.a.36889","article-title":"Multi-systemic involvement in NGLY1-related disorder caused by two novel mutations","volume":"167A","author":"Heeley","year":"2015","journal-title":"Am. J. Med. Genet. A"},{"issue":"7\u20138","key":"10.1016\/j.mam.2016.05.004_bib0360","first-page":"471","article-title":"Biological roles of the non-integrin elastin\/laminin receptor","volume":"377","author":"Hinek","year":"1996","journal-title":"Biol. Chem"},{"issue":"11\u201312","key":"10.1016\/j.mam.2016.05.004_bib0365","doi-asserted-by":"crossref","first-page":"894","DOI":"10.1007\/s000180050482","article-title":"Cathepsin A\/protective protein: an unusual lysosomal multifunctional protein","volume":"56","author":"Hiraiwa","year":"1999","journal-title":"Cell. Mol. Life Sci"},{"key":"10.1016\/j.mam.2016.05.004_bib0370","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/j.semcdb.2014.11.009","article-title":"Physiological and molecular functions of the cytosolic peptide:N-glycanase","volume":"41","author":"Hirayama","year":"2015","journal-title":"Semin. Cell Dev. Biol"},{"issue":"13","key":"10.1016\/j.mam.2016.05.004_bib0375","doi-asserted-by":"crossref","first-page":"3963","DOI":"10.1021\/bi9524575","article-title":"Direct determination of the substrate specificity of the alpha-active site in heterodimeric beta-hexosaminidase A","volume":"35","author":"Hou","year":"1996","journal-title":"Biochemistry"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0380","doi-asserted-by":"crossref","first-page":"1398","DOI":"10.1073\/pnas.1414593112","article-title":"Endo-beta-N-acetylglucosaminidase forms N-GlcNAc protein aggregates during ER-associated degradation in Ngly1-defective cells","volume":"112","author":"Huang","year":"2015","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0385","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1080\/09168451.2014.991682","article-title":"Autophagy regulates the stability of sialin, a lysosomal sialic acid transporter","volume":"79","author":"Huang","year":"2015","journal-title":"Biosci. Biotechnol. Biochem"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0390","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1016\/0005-2744(75)90216-8","article-title":"Specificity studies on alpha-mannosidases using oligosaccharides from mannosidosis urine as substrates","volume":"410","author":"Hultberg","year":"1975","journal-title":"Biochim. Biophys. Acta"},{"issue":"24","key":"10.1016\/j.mam.2016.05.004_bib0395","doi-asserted-by":"crossref","first-page":"11222","DOI":"10.1073\/pnas.88.24.11222","article-title":"Spectrum of mutations in aspartylglucosaminuria","volume":"88","author":"Ikonen","year":"1991","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0400","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1002\/j.1460-2075.1991.tb07920.x","article-title":"Aspartylglucosaminuria: cDNA encoding human aspartylglucosaminidase and the missense mutation causing the disease","volume":"10","author":"Ikonen","year":"1991","journal-title":"EMBO J."},{"issue":"13","key":"10.1016\/j.mam.2016.05.004_bib0405","doi-asserted-by":"crossref","first-page":"8715","DOI":"10.1016\/S0021-9258(19)50336-2","article-title":"Deletion of the 3'-untranslated region of aspartylglucosaminidase mRNA results in a lysosomal accumulation disease","volume":"267","author":"Ikonen","year":"1992","journal-title":"J. Biol. Chem"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0410","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/j.1460-2075.1993.tb05656.x","article-title":"Lysosomal aspartylglucosaminidase is processed to the active subunit complex in the endoplasmic reticulum","volume":"12","author":"Ikonen","year":"1993","journal-title":"EMBO J."},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0415","first-page":"639","article-title":"Characterization of Hex S, the major residual beta hexosaminidase activity in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease)","volume":"27","author":"Ikonne","year":"1975","journal-title":"Am. J. Hum. Genet"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0420","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1042\/BJ20071562","article-title":"Accumulation of free complex-type N-glycans in MKN7 and MKN45 stomach cancer cells","volume":"413","author":"Ishizuka","year":"2008","journal-title":"Biochem. J."},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0425","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1002\/humu.1380050408","article-title":"Identification of a novel mutation causing aspartylglucosaminuria reveals a mutation hotspot region in the aspartylglucosaminidase gene","volume":"5","author":"Isoniemi","year":"1995","journal-title":"Hum. Mutat"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0430","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1042\/bj2680041","article-title":"N-acetyl-D-glucosamine countertransport in lysosomal membrane vesicles","volume":"268","author":"Jonas","year":"1990","journal-title":"Biochem. J."},{"issue":"9","key":"10.1016\/j.mam.2016.05.004_bib0435","doi-asserted-by":"crossref","first-page":"4953","DOI":"10.1016\/S0021-9258(18)83683-3","article-title":"Transport of N-acetyl-D-glucosamine and N-acetyl-D-galactosamine by rat liver lysosomes","volume":"264","author":"Jonas","year":"1989","journal-title":"J. Biol. Chem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0440","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1042\/bj2720323","article-title":"Neutral-sugar transport by rat liver lysosomes","volume":"272","author":"Jonas","year":"1990","journal-title":"Biochem. J."},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib0445","doi-asserted-by":"crossref","first-page":"5185","DOI":"10.1016\/S0021-9258(19)69384-1","article-title":"Caprine beta-mannosidosis. Inherited deficiency of beta-D-mannosidase","volume":"256","author":"Jones","year":"1981","journal-title":"J. Biol. Chem"},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib0450","doi-asserted-by":"crossref","first-page":"5181","DOI":"10.1016\/S0021-9258(19)69383-X","article-title":"Caprine oligosaccharide storage disease. Accumulation of beta-mannosyl (1 goes to 4) beta-N-acetylglucosaminyl (1 goes to 4) beta-N-acetylglucosamine in brain","volume":"256","author":"Jones","year":"1981","journal-title":"J. Biol. Chem"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0455","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1097\/00005072-198305000-00005","article-title":"Caprine beta-mannosidosis: clinical and pathological features","volume":"42","author":"Jones","year":"1983","journal-title":"J. Neuropathol. Exp. Neurol"},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib0460","doi-asserted-by":"crossref","first-page":"6855","DOI":"10.1016\/S0021-9258(19)50505-1","article-title":"Substrate specificity and reaction mechanism of human glycoasparaginase. The N-glycosidic linkage of various glycoasparagines is cleaved through a reaction mechanism similar to L-asparaginase","volume":"267","author":"Kaartinen","year":"1992","journal-title":"J. Biol. Chem"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib0465","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1038\/nm1296-1375","article-title":"A mouse model for the human lysosomal disease aspartylglycosaminuria","volume":"2","author":"Kaartinen","year":"1996","journal-title":"Nat. Med"},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib0470","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1093\/glycob\/cwf073","article-title":"Identification of an endo-beta-N-acetylglucosaminidase gene in Caenorhabditis elegans and its expression in Escherichia coli","volume":"12","author":"Kato","year":"2002","journal-title":"Glycobiology"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0475","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1104\/pp.112.208900","article-title":"Identification and origin of N-linked beta-D-N-acetylglucosamine monosaccharide modifications on Arabidopsis proteins","volume":"161","author":"Kim","year":"2013","journal-title":"Plant Physiol"},{"issue":"9","key":"10.1016\/j.mam.2016.05.004_bib0480","doi-asserted-by":"crossref","first-page":"1255","DOI":"10.1016\/S0022-2275(20)40540-1","article-title":"Saposins: structure, function, distribution, and molecular genetics","volume":"33","author":"Kishimoto","year":"1992","journal-title":"J. Lipid Res"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib0485","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1203\/00006450-199606000-00022","article-title":"Cathepsin A deficiency in galactosialidosis: studies of patients and carriers in 16 families","volume":"39","author":"Kleijer","year":"1996","journal-title":"Pediatr. Res"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0490","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/j.ejcb.2009.10.008","article-title":"Mannose phosphorylation in health and disease","volume":"89","author":"Kollmann","year":"2010","journal-title":"Eur. J. Cell Biol"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0495","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1542\/peds.57.2.205","article-title":"Fucosidosis type 2","volume":"57","author":"Kousseff","year":"1976","journal-title":"Pediatrics"},{"issue":"24","key":"10.1016\/j.mam.2016.05.004_bib0500","doi-asserted-by":"crossref","first-page":"12926","DOI":"10.1016\/S0021-9258(18)42985-7","article-title":"Liberation of N-acetylglucosamine-6-sulfate by human beta-N-acetylhexosaminidase A","volume":"256","author":"Kresse","year":"1981","journal-title":"J. Biol. Chem"},{"issue":"13","key":"10.1016\/j.mam.2016.05.004_bib0505","doi-asserted-by":"crossref","first-page":"5803","DOI":"10.1016\/S0021-9258(17)38454-5","article-title":"A di-N-acetylchitobiase activity is involved in the lysosomal catabolism of asparagine-linked glycoproteins in rat liver","volume":"261","author":"Kuranda","year":"1986","journal-title":"J. Biol. Chem"},{"issue":"23","key":"10.1016\/j.mam.2016.05.004_bib0510","doi-asserted-by":"crossref","first-page":"12316","DOI":"10.1016\/S0021-9258(18)43273-5","article-title":"Four positional isomers of sialyloligosaccharides isolated from the urine of a patient with sialidosis","volume":"256","author":"Kuriyama","year":"1981","journal-title":"J. Biol. Chem"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib0515","doi-asserted-by":"crossref","first-page":"7568","DOI":"10.1016\/S0021-9258(17)39645-X","article-title":"Evidence for two different active sites on human beta-hexosaminidase A. Interaction of GM2 activator protein with beta-hexosaminidase A","volume":"260","author":"Kytzia","year":"1985","journal-title":"J. Biol. Chem"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0520","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1111\/tra.12358","article-title":"N-glycan based ER molecular chaperone and protein quality control system: the calnexin binding cycle","volume":"17","author":"Lamriben","year":"2016","journal-title":"Traffic"},{"issue":"21","key":"10.1016\/j.mam.2016.05.004_bib0525","doi-asserted-by":"crossref","first-page":"7512","DOI":"10.1016\/S0021-9258(19)43319-X","article-title":"Hydrolysis of Tay-Sachs ganglioside by beta-hexosaminidase A of human liver and urine","volume":"248","author":"Li","year":"1973","journal-title":"J. Biol. Chem"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib0530","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1093\/glycob\/9.6.589","article-title":"Structure of the human gene for lysosomal di-N-acetylchitobiase","volume":"9","author":"Liu","year":"1999","journal-title":"Glycobiology"},{"issue":"Database issue","key":"10.1016\/j.mam.2016.05.004_bib0535","doi-asserted-by":"crossref","first-page":"D490","DOI":"10.1093\/nar\/gkt1178","article-title":"The carbohydrate-active enzymes database (CAZy) in 2013","volume":"42","author":"Lombard","year":"2014","journal-title":"Nucleic Acids Res"},{"issue":"1\u20132","key":"10.1016\/j.mam.2016.05.004_bib0540","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1007\/BF00684928","article-title":"Distribution of central nervous system lesions in beta-mannosidosis","volume":"62","author":"Lovell","year":"1983","journal-title":"Acta Neuropathol"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0545","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1042\/bj1930811","article-title":"Degradation of keratan sulphate by beta-N-acetylhexosaminidases A and B","volume":"193","author":"Ludolph","year":"1981","journal-title":"Biochem. J."},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0550","doi-asserted-by":"crossref","first-page":"2326","DOI":"10.1074\/jbc.M109.045302","article-title":"The Neurospora peptide:N-glycanase ortholog PNG1 is essential for cell polarity despite its lack of enzymatic activity","volume":"285","author":"Maerz","year":"2010","journal-title":"J. Biol. Chem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0555","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/0925-4439(91)90044-A","article-title":"The biochemistry of HEXA and HEXB gene mutations causing GM2 gangliosidosis","volume":"1096","author":"Mahuran","year":"1991","journal-title":"Biochim. Biophys. Acta"},{"issue":"2\u20133","key":"10.1016\/j.mam.2016.05.004_bib0560","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/S0925-4439(99)00074-5","article-title":"Biochemical consequences of mutations causing the GM2 gangliosidoses","volume":"1455","author":"Mahuran","year":"1999","journal-title":"Biochim. Biophys. Acta"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0565","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1093\/oxfordjournals.jbchem.a128760","article-title":"Studies on enzymes acting on glycopeptides","volume":"63","author":"Makino","year":"1968","journal-title":"J. Biochem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0570","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1007\/BF00697387","article-title":"Beta-mannosidosis: lesions of the distal peripheral nervous system","volume":"61","author":"Malachowski","year":"1983","journal-title":"Acta Neuropathol"},{"key":"10.1016\/j.mam.2016.05.004_bib0575","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1186\/1750-1172-3-21","article-title":"Alpha-mannosidosis","volume":"3","author":"Malm","year":"2008","journal-title":"Orphanet J. Rare Dis"},{"issue":"26","key":"10.1016\/j.mam.2016.05.004_bib0580","doi-asserted-by":"crossref","first-page":"15247","DOI":"10.1016\/S0021-9258(19)84817-2","article-title":"Characterization of a proton-driven carrier for sialic acid in the lysosomal membrane. Evidence for a group-specific transport system for acidic monosaccharides","volume":"264","author":"Mancini","year":"1989","journal-title":"J. Biol. Chem"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0585","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1042\/bj2220261","article-title":"Cobalt-ion chelate affinity chromatography for the purification of brain neutral alpha-D-mannosidase and its separation from acid alpha-D-mannosidase","volume":"222","author":"Mathur","year":"1984","journal-title":"Biochem. J."},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0590","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/S0387-7604(96)00077-0","article-title":"Neurological manifestations of knockout mice with beta-galactosidase deficiency","volume":"19","author":"Matsuda","year":"1997","journal-title":"Brain Dev"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib0595","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1023\/A:1018573518127","article-title":"Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis","volume":"14","author":"Matsuda","year":"1997","journal-title":"Glycoconj. J."},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0600","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/0009-8981(80)90016-9","article-title":"N-Acetylglucosamine-asparagine levels in tissues of patients with aspartylglycosaminuria","volume":"108","author":"Maury","year":"1980","journal-title":"Clin. Chim. Acta"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0605","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1007\/BF02179139","article-title":"Aspartylglycosaminuria \u2013 an inborn error of glycoprotein catabolism","volume":"5","author":"Maury","year":"1982","journal-title":"J. Inherit. Metab. Dis"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0610","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/0167-4838(91)90049-6","article-title":"A study of the heterogenous structure of guinea pig lysosomal beta-mannosidase using a polyclonal antibody","volume":"1077","author":"McCabe","year":"1991","journal-title":"Biochim. Biophys. Acta"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0615","doi-asserted-by":"crossref","first-page":"1879","DOI":"10.1016\/S0021-9258(18)52375-9","article-title":"The human GM2 activator protein. A substrate specific cofactor of beta-hexosaminidase A","volume":"266","author":"Meier","year":"1991","journal-title":"J. Biol. Chem"},{"key":"10.1016\/j.mam.2016.05.004_bib0620","series-title":"Glycoproteins and Disease","first-page":"55","article-title":"Normal and pathological catabolism of glycoproteins. Catabolic pathways","author":"Michalski","year":"1996"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0625","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/0014-5793(77)80359-1","article-title":"Structures of sialyl-oligosaccharides excreted in the urine of a patient with mucolipidosis I","volume":"79","author":"Michalski","year":"1977","journal-title":"FEBS Lett"},{"key":"10.1016\/j.mam.2016.05.004_bib0630","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1016\/S0008-6215(00)81048-4","article-title":"The structures of six urinary oligosaccharides that are characteristic for a patient with Morquio syndrome type B","volume":"100","author":"Michalski","year":"1982","journal-title":"Carbohydr. Res"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0635","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1111\/j.1432-1033.1990.tb15498.x","article-title":"In vitro hydrolysis of oligomannosyl oligosaccharides by the lysosomal alpha-D-mannosidases","volume":"189","author":"Michalski","year":"1990","journal-title":"Eur. J. Biochem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0640","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1111\/j.1432-1033.1991.tb16302.x","article-title":"Characterization and 400-MHz 1H-NMR analysis of urinary fucosyl glycoasparagines in fucosidosis","volume":"201","author":"Michalski","year":"1991","journal-title":"Eur. J. Biochem"},{"issue":"7","key":"10.1016\/j.mam.2016.05.004_bib0645","doi-asserted-by":"crossref","first-page":"4549","DOI":"10.1074\/jbc.272.7.4549","article-title":"Identification of a sialidase encoded in the human major histocompatibility complex","volume":"272","author":"Milner","year":"1997","journal-title":"J. Biol. Chem"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0650","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1111\/j.1432-1033.1984.tb08159.x","article-title":"Rat-liver lysosomal sialidase. Solubilization, substrate specificity and comparison with the cytosolic sialidase","volume":"141","author":"Miyagi","year":"1984","journal-title":"Eur. J. Biochem"},{"issue":"33","key":"10.1016\/j.mam.2016.05.004_bib0655","doi-asserted-by":"crossref","first-page":"11720","DOI":"10.1073\/pnas.0804015105","article-title":"Identification of a vesicular aspartate transporter","volume":"105","author":"Miyaji","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"13","key":"10.1016\/j.mam.2016.05.004_bib0660","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.1096\/fasebj.7.13.8405810","article-title":"Aspartylglycosaminuria \u2013 protein chemistry and molecular-biology of the most common lysosomal storage disorder of glycoprotein degradation","volume":"7","author":"Mononen","year":"1993","journal-title":"FASEB J."},{"issue":"34","key":"10.1016\/j.mam.2016.05.004_bib0665","doi-asserted-by":"crossref","first-page":"20655","DOI":"10.1016\/S0021-9258(19)47114-7","article-title":"Alternative splicing of beta-galactosidase mRNA generates the classic lysosomal enzyme and a beta-galactosidase-related protein","volume":"264","author":"Morreau","year":"1989","journal-title":"J. Biol. Chem"},{"issue":"17","key":"10.1016\/j.mam.2016.05.004_bib0670","doi-asserted-by":"crossref","first-page":"5394","DOI":"10.1073\/pnas.81.17.5394","article-title":"cDNA clone for the alpha-chain of human beta-hexosaminidase: deficiency of alpha-chain mRNA in Ashkenazi Tay-Sachs fibroblasts","volume":"81","author":"Myerowitz","year":"1984","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"8","key":"10.1016\/j.mam.2016.05.004_bib0675","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1093\/glycob\/cwu033","article-title":"Structural change of N-glycan exposes hydrophobic surface of human transferrin","volume":"24","author":"Nagae","year":"2014","journal-title":"Glycobiology"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0680","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1006\/bbrc.1994.1440","article-title":"Human lysosomal alpha-mannosidase: isolation and nucleotide sequence of the full-length cDNA","volume":"200","author":"Nebes","year":"1994","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib0685","doi-asserted-by":"crossref","first-page":"353","DOI":"10.1136\/jmedgenet-2012-100819","article-title":"Clinical application of exome sequencing in undiagnosed genetic conditions","volume":"49","author":"Need","year":"2012","journal-title":"J. Med. Genet"},{"key":"10.1016\/j.mam.2016.05.004_bib0690","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1146\/annurev.bi.60.070191.001353","article-title":"Lysosomal storage diseases","volume":"60","author":"Neufeld","year":"1991","journal-title":"Annu. Rev. Biochem"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0695","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1016\/S0006-291X(74)80055-0","article-title":"Oligosaccharides accumulating in the liver from a patient with GM2-gangliosidosis variant O (Sandhoff-Jatzkewitz disease)","volume":"59","author":"Ng-Ying-Kin","year":"1974","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0700","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/S0006-291X(75)80303-2","article-title":"Characterization of oligosaccharides and glycopeptides excreted in the urine of GMl-gangliosidosis patients","volume":"66","author":"Ng-Ying-Kin","year":"1975","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0705","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1016\/S0006-291X(74)80027-6","article-title":"Endoglycosidases acting on carbohydrate moieties of glycoproteins: demonstration in mammalian tissue","volume":"59","author":"Nishigaki","year":"1974","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib0710","doi-asserted-by":"crossref","first-page":"1645","DOI":"10.1515\/bchm2.1983.364.2.1645","article-title":"beta-D-Mannosidase from human placenta: properties and partial purification","volume":"364","author":"Noeske","year":"1983","journal-title":"Hoppe Seylers Z. Physiol. Chem"},{"issue":"17","key":"10.1016\/j.mam.2016.05.004_bib0715","doi-asserted-by":"crossref","first-page":"6210","DOI":"10.1016\/S0021-9258(19)43529-1","article-title":"A mannose-containing trisaccharide isolated from urines of three patients with mannosidosis","volume":"248","author":"Norden","year":"1973","journal-title":"J. Biol. Chem"},{"key":"10.1016\/j.mam.2016.05.004_bib9005","doi-asserted-by":"crossref","first-page":"7969","DOI":"10.1016\/S0021-9258(19)42060-7","article-title":"GM1 ganglioside beta-galactosidase. A. Purification and studies of the enzyme from human liver","volume":"249","author":"Norden","year":"1974","journal-title":"J. Biol. Chem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0720","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1093\/glycob\/7.2.217","article-title":"Influence of L-fucose attached alpha 1\u21926 to the asparagine-linked N-acetylglucosamine on the hydrolysis of the N-glycosidic linkage by human glycosylasparaginase","volume":"7","author":"Noronkoski","year":"1997","journal-title":"Glycobiology"},{"key":"10.1016\/j.mam.2016.05.004_bib0725","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1001\/archpedi.1965.02090020340014","article-title":"Generalized gangliosidosis: another inborn error of ganglioside metabolism?","volume":"109","author":"O'Brien","year":"1965","journal-title":"Am. J. Dis. Child"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0730","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/0006-291X(89)91739-7","article-title":"Human alpha-L-fucosidase: complete coding sequence from cDNA clones","volume":"164","author":"Occhiodoro","year":"1989","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"7509","key":"10.1016\/j.mam.2016.05.004_bib0735","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1016\/S0140-6736(67)92303-3","article-title":"A generalised storage disorder resembling Hurler's syndrome","volume":"2","author":"Ockerman","year":"1967","journal-title":"Lancet"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0740","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1073\/pnas.82.4.1184","article-title":"Isolation of cDNA clones coding for the beta subunit of human beta-hexosaminidase","volume":"82","author":"O'Dowd","year":"1985","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0745","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1093\/oxfordjournals.jbchem.a022526","article-title":"Quantitation and isomeric structure analysis of free oligosaccharides present in the cytosol fraction of mouse liver: detection of a free disialobiantennary oligosaccharide and glucosylated oligomannosides","volume":"126","author":"Ohashi","year":"1999","journal-title":"J. Biochem"},{"issue":"16","key":"10.1016\/j.mam.2016.05.004_bib0750","doi-asserted-by":"crossref","first-page":"8485","DOI":"10.1016\/S0021-9258(19)68869-1","article-title":"Urinary oligosaccharides of GM1-gangliosidosis. Structures of oligosaccharides excreted in the urine of type 1 but not in the urine of type 2 patients","volume":"256","author":"Ohkura","year":"1981","journal-title":"J. Biol. Chem"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib0755","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1038\/nsb1295-1102","article-title":"Three-dimensional structure of human lysosomal aspartylglucosaminidase","volume":"2","author":"Oinonen","year":"1995","journal-title":"Nat. Struct. Biol"},{"key":"10.1016\/j.mam.2016.05.004_bib9000","doi-asserted-by":"crossref","first-page":"1002","DOI":"10.1126\/science.160.3831.1002","article-title":"Generalized gangliosidosis: beta-galactosidase deficiency","volume":"160","author":"Okada","year":"1968","journal-title":"Science"},{"issue":"3894","key":"10.1016\/j.mam.2016.05.004_bib0760","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1126\/science.165.3894.698","article-title":"Tay-Sachs disease: generalized absence of a beta-D-N-acetylhexosaminidase component","volume":"165","author":"Okada","year":"1969","journal-title":"Science"},{"issue":"7","key":"10.1016\/j.mam.2016.05.004_bib0765","first-page":"606","article-title":"Sandhoff's disease (GM 2 gangliosidosis type 2): clinical, chemical, and enzyme studies in five patients","volume":"6","author":"Okada","year":"1972","journal-title":"Pediatr. Res"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib0770","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1093\/oxfordjournals.jbchem.a123700","article-title":"Studies on the substrate specificity of neutral alpha-mannosidase purified from Japanese quail oviduct by using sugar chains from glycoproteins","volume":"110","author":"Oku","year":"1991","journal-title":"J. Biochem"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0775","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1016\/S0006-291X(88)80038-X","article-title":"Cloning, sequencing, and expression of cDNA for human beta-galactosidase","volume":"157","author":"Oshima","year":"1988","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"14","key":"10.1016\/j.mam.2016.05.004_bib0780","doi-asserted-by":"crossref","first-page":"9611","DOI":"10.1074\/jbc.M114.550509","article-title":"Accumulation of free oligosaccharides and tissue damage in cytosolic alpha-mannosidase (Man2c1)-deficient mice","volume":"289","author":"Paciotti","year":"2014","journal-title":"J. Biol. Chem"},{"issue":"44","key":"10.1016\/j.mam.2016.05.004_bib0785","doi-asserted-by":"crossref","first-page":"37204","DOI":"10.1074\/jbc.M508930200","article-title":"Characterization of a human core-specific lysosomal alpha1,6-mannosidase involved in N-glycan catabolism","volume":"280","author":"Park","year":"2005","journal-title":"J. Biol. Chem"},{"issue":"4033","key":"10.1016\/j.mam.2016.05.004_bib0790","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1126\/science.176.4033.426","article-title":"Deficiency of alpha-L-fucosidase","volume":"176","author":"Patel","year":"1972","journal-title":"Science"},{"issue":"7","key":"10.1016\/j.mam.2016.05.004_bib0795","doi-asserted-by":"crossref","first-page":"1137","DOI":"10.1016\/j.bbadis.2012.03.005","article-title":"Lysosomal di-N-acetylchitobiase-deficient mouse tissues accumulate Man2GlcNAc2 and Man3GlcNAc2","volume":"1822","author":"Persichetti","year":"2012","journal-title":"Biochim. Biophys. Acta"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0800","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1042\/bj1800673","article-title":"Cytosolic location of an endo-N-acetyl-beta-D-glucosaminidase activity in rat liver and kidney","volume":"180","author":"Pierce","year":"1979","journal-title":"Biochem. J."},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0805","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1042\/bj1410141","article-title":"The glycoasparagines in urine of a patient with aspartylglycosaminuria","volume":"141","author":"Pollitt","year":"1974","journal-title":"Biochem. J."},{"issue":"7562","key":"10.1016\/j.mam.2016.05.004_bib0810","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/S0140-6736(68)92355-6","article-title":"Aspartylglycosaminuria. An inborn error of metabolism associated with mental defect","volume":"2","author":"Pollitt","year":"1968","journal-title":"Lancet"},{"issue":"11","key":"10.1016\/j.mam.2016.05.004_bib0815","doi-asserted-by":"crossref","first-page":"6319","DOI":"10.1074\/jbc.273.11.6319","article-title":"The 67-kDa enzymatically inactive alternatively spliced variant of beta-galactosidase is identical to the elastin\/laminin-binding protein","volume":"273","author":"Privitera","year":"1998","journal-title":"J. Biol. Chem"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0820","doi-asserted-by":"crossref","first-page":"3350","DOI":"10.1016\/S0021-9258(17)43301-1","article-title":"Association of alpha- and beta-subunits during the biosynthesis of beta-hexosaminidase in cultured human fibroblasts","volume":"259","author":"Proia","year":"1984","journal-title":"J. Biol. Chem"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0825","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1038\/ng0397-316","article-title":"Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis","volume":"15","author":"Pshezhetsky","year":"1997","journal-title":"Nat. Genet"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0830","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1042\/bj1070321","article-title":"N-Acetyl-beta-glucosaminidases in human spleen","volume":"107","author":"Robinson","year":"1968","journal-title":"Biochem. J."},{"key":"10.1016\/j.mam.2016.05.004_bib0835","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1097\/00005053-188709000-00001","article-title":"On arrested cerebral development with special reference to its cortical pathology","volume":"14","author":"Sachs","year":"1887","journal-title":"J. Nerv. Ment. Dis"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0840","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1083\/jcb.136.1.45","article-title":"Transfer of free polymannose-type oligosaccharides from the cytosol to lysosomes in cultured human hepatocellular carcinoma HepG2 cells","volume":"136","author":"Saint-Pol","year":"1997","journal-title":"J. Cell Biol"},{"issue":"19","key":"10.1016\/j.mam.2016.05.004_bib0845","doi-asserted-by":"crossref","first-page":"13547","DOI":"10.1074\/jbc.274.19.13547","article-title":"Cytosol-to-lysosome transport of free polymannose-type oligosaccharides. Kinetic and specificity studies using rat liver lysosomes","volume":"274","author":"Saint-Pol","year":"1999","journal-title":"J. Biol. Chem"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0850","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1016\/0014-5793(69)80274-7","article-title":"Variation of beta-N-acetylhexosaminidase-pattern in Tay-Sachs disease","volume":"4","author":"Sandhoff","year":"1969","journal-title":"FEBS Lett"},{"issue":"8","key":"10.1016\/j.mam.2016.05.004_bib0855","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1515\/bchm2.1971.352.2.1119","article-title":"[Enrichment and characterization of 2 forms of human N-acetyl-beta-D-hexosaminidase]","volume":"352","author":"Sandhoff","year":"1971","journal-title":"Hoppe Seylers Z. Physiol. Chem"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib0860","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/0024-3205(68)90024-6","article-title":"Deficient hexozaminidase activity in an exceptional case of Tay-Sachs disease with additional storage of kidney globoside in visceral organs","volume":"7","author":"Sandhoff","year":"1968","journal-title":"Life Sci"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0865","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/ng1095-170","article-title":"Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism","volume":"11","author":"Sango","year":"1995","journal-title":"Nat. Genet"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0870","doi-asserted-by":"crossref","first-page":"2475","DOI":"10.3390\/molecules20022475","article-title":"Emerging structural insights into glycoprotein quality control coupled with N-glycan processing in the endoplasmic reticulum","volume":"20","author":"Satoh","year":"2015","journal-title":"Molecules"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib0875","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1515\/bchm3.1991.372.1.443","article-title":"Lysosomal and plasma membrane ganglioside GM3 sialidases of cultured human fibroblasts. Differentiation by detergents and inhibitors","volume":"372","author":"Schneider-Jakob","year":"1991","journal-title":"Biol. Chem. Hoppe Seyler"},{"issue":"11","key":"10.1016\/j.mam.2016.05.004_bib0880","doi-asserted-by":"crossref","first-page":"4352","DOI":"10.1091\/mbc.e02-07-0433","article-title":"The genetic basis of cellular morphogenesis in the filamentous fungus Neurospora crassa","volume":"14","author":"Seiler","year":"2003","journal-title":"Mol. Biol. Cell"},{"issue":"37","key":"10.1016\/j.mam.2016.05.004_bib0885","doi-asserted-by":"crossref","first-page":"26898","DOI":"10.1074\/jbc.M113.464503","article-title":"Basal autophagy is required for the efficient catabolism of sialyloligosaccharides","volume":"288","author":"Seino","year":"2013","journal-title":"J. Biol. Chem"},{"key":"10.1016\/j.mam.2016.05.004_bib0890","doi-asserted-by":"crossref","DOI":"10.1093\/glycob\/cww048","article-title":"Occurrence of free sialyl oligosaccharides related to N-glycans (sialyl FNGs) in animal sera","volume":"26","author":"Seino","year":"2016","journal-title":"Glycobiology"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0895","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1002\/humu.10268","article-title":"Molecular pathology of NEU1 gene in sialidosis","volume":"22","author":"Seyrantepe","year":"2003","journal-title":"Hum. Mutat"},{"issue":"13","key":"10.1016\/j.mam.2016.05.004_bib0900","doi-asserted-by":"crossref","first-page":"3845","DOI":"10.1016\/S0021-9258(17)33324-0","article-title":"Purification and characterization of the alpha-D-mannosidase of rat liver cytosol","volume":"251","author":"Shoup","year":"1976","journal-title":"J. Biol. Chem"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib0905","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1542\/peds.72.4.441","article-title":"Sialic acid storage disease with sialuria: clinical and biochemical features in the severe infantile type","volume":"72","author":"Stevenson","year":"1983","journal-title":"Pediatrics"},{"issue":"8","key":"10.1016\/j.mam.2016.05.004_bib0910","doi-asserted-by":"crossref","first-page":"1365","DOI":"10.1093\/hmg\/8.8.1365","article-title":"Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis","volume":"8","author":"Stinchi","year":"1999","journal-title":"Hum. Mol. Genet"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0915","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/0014-5793(74)80044-X","article-title":"Human N-acetyl-beta-hexosaminidases: hydrolysis of N, N' diacetylchitobiose by a low molecular weight enzyme","volume":"39","author":"Stirling","year":"1974","journal-title":"FEBS Lett"},{"issue":"11\u201312","key":"10.1016\/j.mam.2016.05.004_bib0920","first-page":"1199","article-title":"[Glycoproteins and glycoproteinosis]","volume":"61","author":"Strecker","year":"1979","journal-title":"Biochimie"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0925","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1016\/S0300-9084(76)80227-1","article-title":"[Chemistry of urinary mannosides excreted in mannosidosis]","volume":"58","author":"Strecker","year":"1976","journal-title":"Biochimie"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0930","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1111\/j.1432-1033.1977.tb11937.x","article-title":"Structure of seven oligosaccharides excreted in the urine of a patient with Sandhoff's disease (GM2 gangliosidosis-variant O)","volume":"81","author":"Strecker","year":"1977","journal-title":"Eur. J. Biochem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0935","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1111\/j.1432-1033.1977.tb11540.x","article-title":"Structure of nine sialyl-oligosaccharides accumulatedin urine of eleven patients with three different types of sialidosis. Mucolipidosis II and two new types of mucolipidosis","volume":"75","author":"Strecker","year":"1977","journal-title":"Eur. J. Biochem"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0940","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/0014-5793(77)80324-4","article-title":"Structural studies of glycoasparagines from urine of a patient with aspartylglycosylaminuria (AGU)","volume":"78","author":"Sugahara","year":"1977","journal-title":"FEBS Lett"},{"issue":"8","key":"10.1016\/j.mam.2016.05.004_bib0945","doi-asserted-by":"crossref","first-page":"1493","DOI":"10.1021\/bi00758a026","article-title":"Purification and properties of alpha-D- and beta-D-mannosidases from hen oviduct","volume":"11","author":"Sukeno","year":"1972","journal-title":"Biochemistry"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib0950","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1016\/j.semcdb.2007.09.010","article-title":"Cytoplasmic peptide:N-glycanase and catabolic pathway for free N-glycans in the cytosol","volume":"18","author":"Suzuki","year":"2007","journal-title":"Semin. Cell Dev. Biol"},{"issue":"120","key":"10.1016\/j.mam.2016.05.004_bib0955","doi-asserted-by":"crossref","first-page":"219","DOI":"10.4052\/tigg.21.219","article-title":"Introduction to \u201cGlycometabolome\u201d","volume":"21","author":"Suzuki","year":"2009","journal-title":"Trends Glycosci. Glycotechnol"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib0960","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1093\/jb\/mvu068","article-title":"The cytoplasmic peptide:N-glycanase (Ngly1)-basic science encounters a human genetic disorder","volume":"157","author":"Suzuki","year":"2015","journal-title":"J. Biochem"},{"issue":"5\u20136","key":"10.1016\/j.mam.2016.05.004_bib0965","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1007\/s10719-006-6975-x","article-title":"Free N-linked oligosaccharide chains: formation and degradation","volume":"23","author":"Suzuki","year":"2006","journal-title":"Glycoconj. J."},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib0970","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.bbrc.2014.05.075","article-title":"Non-lysosomal degradation pathway for N-linked glycans and dolichol-linked oligosaccharides","volume":"453","author":"Suzuki","year":"2014","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib0975","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.1006\/bbrc.1993.1938","article-title":"Identification of peptide:N-glycanase activity in mammalian-derived cultured cells","volume":"194","author":"Suzuki","year":"1993","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"26","key":"10.1016\/j.mam.2016.05.004_bib0980","doi-asserted-by":"crossref","first-page":"17611","DOI":"10.1016\/S0021-9258(17)32485-7","article-title":"Purification and enzymatic-properties of peptide-N-glycanase from C3h mouse-derived L-929 fibroblast cells \u2013 possible widespread occurrence of posttranslational, remodification of proteins by N-deglycosylation","volume":"269","author":"Suzuki","year":"1994","journal-title":"J. Biol. Chem"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib0985","doi-asserted-by":"crossref","first-page":"1039","DOI":"10.1083\/jcb.149.5.1039","article-title":"PNG1, a yeast gene encoding a highly conserved peptide:N-glycanase","volume":"149","author":"Suzuki","year":"2000","journal-title":"J. Cell Biol"},{"issue":"7","key":"10.1016\/j.mam.2016.05.004_bib0990","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1096\/fj.01-0889rev","article-title":"Cytoplasmic peptide: N-glycanase (PNGase) in eukaryotic cells: occurrence, primary structure, and potential functions","volume":"16","author":"Suzuki","year":"2002","journal-title":"FASEB J."},{"issue":"15","key":"10.1016\/j.mam.2016.05.004_bib0995","doi-asserted-by":"crossref","first-page":"9691","DOI":"10.1073\/pnas.152333599","article-title":"Endo-beta-N-acetylglucosaminidase, an enzyme involved in processing of free oligosaccharides in the cytosol","volume":"99","author":"Suzuki","year":"2002","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib1000","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1042\/BJ20060945","article-title":"Man2C1, an alpha-mannosidase, is involved in the trimming of free oligosaccharides in the cytosol","volume":"400","author":"Suzuki","year":"2006","journal-title":"Biochem. J."},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib1005","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.gene.2015.11.021","article-title":"The cytoplasmic peptide:N-glycanase (NGLY1) \u2013 Structure, expression and cellular functions","volume":"577","author":"Suzuki","year":"2016","journal-title":"Gene"},{"key":"10.1016\/j.mam.2016.05.004_bib1010","series-title":"The Metabolic and Molecular Bases of Inherited Disease","first-page":"3775","article-title":"beta-Galactosidase deficiency (beta-galactosidosis) GM1 gangliosidosis and Morquio B disease","author":"Suzuki","year":"2001"},{"key":"10.1016\/j.mam.2016.05.004_bib1015","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1016\/0006-291X(62)90030-X","article-title":"The chemical structure of normal human brain and Tay-Sachs gangliosides","volume":"9","author":"Svennerholm","year":"1962","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib1020","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1093\/oxfordjournals.jbchem.a130202","article-title":"Specificity studies of 4-L-aspartylglycosylamine amido hydrolase","volume":"73","author":"Tanaka","year":"1973","journal-title":"J. Biochem"},{"key":"10.1016\/j.mam.2016.05.004_bib1025","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.semcdb.2014.12.001","article-title":"N-linked sugar-regulated protein folding and quality control in the ER","volume":"41","author":"Tannous","year":"2015","journal-title":"Semin. Cell Dev. Biol"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib1030","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/0003-9861(69)90036-8","article-title":"The purification and properties of a beta-aspartyl N-acetylglucosylamine amidohydrolase from hen oviduct","volume":"130","author":"Tarentino","year":"1969","journal-title":"Arch. Biochem. Biophys"},{"issue":"25","key":"10.1016\/j.mam.2016.05.004_bib1035","doi-asserted-by":"crossref","first-page":"5516","DOI":"10.1021\/bi00696a021","article-title":"The isolation and structure of the core oligosaccharide sequences of IgM","volume":"14","author":"Tarentino","year":"1975","journal-title":"Biochemistry"},{"key":"10.1016\/j.mam.2016.05.004_bib1040","first-page":"155","article-title":"Symmetrical changes in the region of the yellow spot in each eye of an infant","volume":"1","author":"Tay","year":"1881","journal-title":"Trans. Ophthalmol. Soc. U. K."},{"issue":"4102","key":"10.1016\/j.mam.2016.05.004_bib1045","doi-asserted-by":"crossref","first-page":"866","DOI":"10.1126\/science.181.4102.866","article-title":"N-acetyl-beta-hexosaminidase: role in the degradation of glycosaminoglycans","volume":"181","author":"Thompson","year":"1973","journal-title":"Science"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib1050","doi-asserted-by":"crossref","first-page":"2428","DOI":"10.1111\/j.1349-7006.2008.01019.x","article-title":"Inhibition of alpha-mannosidase Man2c1 gene expression suppresses growth of esophageal carcinoma cells through mitotic arrest and apoptosis","volume":"99","author":"Tian","year":"2008","journal-title":"Cancer Sci"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib1055","doi-asserted-by":"crossref","first-page":"2954","DOI":"10.1002\/j.1460-2075.1996.tb00658.x","article-title":"Functional analyses of active site residues of human lysosomal aspartylglucosaminidase: implications for catalytic mechanism and autocatalytic activation","volume":"15","author":"Tikkanen","year":"1996","journal-title":"EMBO J."},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib1060","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1042\/bj2600101","article-title":"Purification and characterization of rat liver glycosylasparaginase","volume":"260","author":"Tollersrud","year":"1989","journal-title":"Biochem. J."},{"issue":"8","key":"10.1016\/j.mam.2016.05.004_bib1065","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1074\/mcp.O112.018366","article-title":"Global identification and characterization of bothO-GlcNAcylation and phosphorylation at the murine synapse","volume":"11","author":"Trinidad","year":"2012","journal-title":"Mol. Cell. Proteomics"},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib1070","doi-asserted-by":"crossref","first-page":"3474","DOI":"10.1074\/mcp.M113.030007","article-title":"N- and O-glycosylation in the murine synaptosome","volume":"12","author":"Trinidad","year":"2013","journal-title":"Mol. Cell. Proteomics"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib1075","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1016\/0006-291X(73)91002-4","article-title":"Structure of the \u201ckeratosulfate-like\u201d material in liver from a patient with G M1 -gangliosidosis (beta-D-galactosidase deficiency)","volume":"52","author":"Tsay","year":"1973","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"19","key":"10.1016\/j.mam.2016.05.004_bib1080","doi-asserted-by":"crossref","first-page":"5852","DOI":"10.1016\/S0021-9258(17)33030-2","article-title":"Structure of the accumulating oligosaccharide in fucosidosis","volume":"251","author":"Tsay","year":"1976","journal-title":"J. Biol. Chem"},{"issue":"33","key":"10.1016\/j.mam.2016.05.004_bib1085","doi-asserted-by":"crossref","first-page":"10894","DOI":"10.1021\/bi960503a","article-title":"Identification of functional domains within the alpha and beta subunits of beta-hexosaminidase A through the expression of alpha-beta fusion proteins","volume":"35","author":"Tse","year":"1996","journal-title":"Biochemistry"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib1090","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1002\/ana.22262","article-title":"Highly phosphomannosylated enzyme replacement therapy for GM2 gangliosidosis","volume":"69","author":"Tsuji","year":"2011","journal-title":"Ann. Neurol"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib1095","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/0003-9861(84)90522-8","article-title":"The similar effects of swainsonine and locoweed on tissue glycosidases and oligosaccharides of the pig indicate that the alkaloid is the principal toxin responsible for the induction of locoism","volume":"232","author":"Tulsiani","year":"1984","journal-title":"Arch. Biochem. Biophys"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib1100","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/j.bbrc.2014.05.017","article-title":"Endo-beta-N-acetylglucosamidases (ENGases) in the fungus Trichoderma atroviride: possible involvement of the filamentous fungi-specific cytosolic ENGase in the ERAD process","volume":"449","author":"Tzelepis","year":"2014","journal-title":"Biochem. Biophys. Res. Commun"},{"issue":"32","key":"10.1016\/j.mam.2016.05.004_bib1105","doi-asserted-by":"crossref","first-page":"19685","DOI":"10.1016\/S0021-9258(17)45426-3","article-title":"Sialyl-alpha 2-6-mannosyl-beta 1-4-N-acetylglucosamine, a novel compound occurring in urine of patients with beta-mannosidosis","volume":"265","author":"van Pelt","year":"1990","journal-title":"J. Biol. Chem"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib1110","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/0009-8981(90)90261-P","article-title":"Accumulation of mannosyl-beta(1\u21924)-N-acetylglucosamine in fibroblasts and leukocytes of patients with a deficiency of beta-mannosidase","volume":"187","author":"van Pelt","year":"1990","journal-title":"Clin. Chim. Acta"},{"issue":"7553","key":"10.1016\/j.mam.2016.05.004_bib1115","doi-asserted-by":"crossref","first-page":"1198","DOI":"10.1016\/S0140-6736(68)91895-3","article-title":"Mucopolysaccharidosis by absence of alpha-fucosidase","volume":"1","author":"Van Hoof","year":"1968","journal-title":"Lancet"},{"issue":"4","key":"10.1016\/j.mam.2016.05.004_bib1120","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1038\/70585","article-title":"A new gene, encoding an anion transporter, is mutated in sialic acid storage diseases","volume":"23","author":"Verheijen","year":"1999","journal-title":"Nat. Genet"},{"issue":"17","key":"10.1016\/j.mam.2016.05.004_bib1125","doi-asserted-by":"crossref","first-page":"11887","DOI":"10.1074\/jbc.M112.425702","article-title":"Dual functions for cytosolic alpha-mannosidase (Man2C1): its down-regulation causes mitochondria-dependent apoptosis independently of its alpha-mannosidase activity","volume":"288","author":"Wang","year":"2013","journal-title":"J. Biol. Chem"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib1130","doi-asserted-by":"crossref","first-page":"1499","DOI":"10.3390\/biom5031499","article-title":"Co-expression of NEU2 and GBA3 causes a drastic reduction in cytosolic sialyl free N-glycans in human MKN45 stomach cancer cells-evidence for the physical interaction of NEU2 and GBA3","volume":"5","author":"Wang","year":"2015","journal-title":"Biomolecules"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib1135","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1016\/S0021-9258(19)68349-3","article-title":"Structure analysis of the major oligosaccharides accumulating in canine GM1 gangliosidosis liver","volume":"257","author":"Warner","year":"1982","journal-title":"J. Biol. Chem"},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib1140","doi-asserted-by":"crossref","first-page":"6194","DOI":"10.1016\/S0021-9258(18)88956-6","article-title":"Characterization and analysis of branched-chain N-acetylglucosaminyl oligosaccharides accumulating in Sandhoff disease tissue. Evidence that biantennary bisected oligosaccharide side chains of glycoproteins are abundant substrates for lysosomes","volume":"260","author":"Warner","year":"1985","journal-title":"J. Biol. Chem"},{"issue":"19","key":"10.1016\/j.mam.2016.05.004_bib1145","doi-asserted-by":"crossref","first-page":"1201","DOI":"10.1056\/NEJM198611063151906","article-title":"Human beta-mannosidase deficiency","volume":"315","author":"Wenger","year":"1986","journal-title":"N. Engl. J. Med"},{"issue":"5","key":"10.1016\/j.mam.2016.05.004_bib1150","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1016\/S0092-8674(00)81054-5","article-title":"The human cytomegalovirus US11 gene product dislocates MHC class I heavy chains from the endoplasmic reticulum to the cytosol","volume":"84","author":"Wiertz","year":"1996","journal-title":"Cell"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib1155","doi-asserted-by":"crossref","DOI":"10.1093\/glycob\/cwi041","article-title":"Lysosomal metabolism of glycoproteins","volume":"15","author":"Winchester","year":"2005","journal-title":"Glycobiology"},{"issue":"6","key":"10.1016\/j.mam.2016.05.004_bib1160","doi-asserted-by":"crossref","first-page":"1828","DOI":"10.1016\/S0021-9258(19)42862-7","article-title":"The structures of oligosaccharides accumulating in the liver of G-M1-gangliosidosis, type I","volume":"249","author":"Wolfe","year":"1974","journal-title":"J. Biol. Chem"},{"issue":"1","key":"10.1016\/j.mam.2016.05.004_bib1165","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1007\/s11248-009-9299-3","article-title":"hMan2c1 transgene promotes tumor progress in mice","volume":"19","author":"Xiang","year":"2010","journal-title":"Transgenic Res"},{"issue":"11","key":"10.1016\/j.mam.2016.05.004_bib1170","doi-asserted-by":"crossref","first-page":"4820","DOI":"10.1016\/S0021-9258(17)30086-8","article-title":"Urinary glycopeptides of fucosidosis","volume":"254","author":"Yamashita","year":"1979","journal-title":"J. Biol. Chem"},{"issue":"11","key":"10.1016\/j.mam.2016.05.004_bib1175","doi-asserted-by":"crossref","first-page":"5126","DOI":"10.1016\/S0021-9258(19)70759-5","article-title":"Urinary oligosaccharides of mannosidosis","volume":"255","author":"Yamashita","year":"1980","journal-title":"J. Biol. Chem"},{"issue":"10","key":"10.1016\/j.mam.2016.05.004_bib1180","doi-asserted-by":"crossref","first-page":"4789","DOI":"10.1016\/S0021-9258(19)69322-1","article-title":"Urinary oligosaccharides of GM1-gangliosidosis. Different excretion patterns of oligosaccharides in the urine of type 1 and type 2 subgroups","volume":"256","author":"Yamashita","year":"1981","journal-title":"J. Biol. Chem"},{"issue":"44","key":"10.1016\/j.mam.2016.05.004_bib1185","doi-asserted-by":"crossref","first-page":"13657","DOI":"10.1073\/pnas.1515464112","article-title":"An intrinsic mechanism of secreted protein aging and turnover","volume":"112","author":"Yang","year":"2015","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"issue":"12","key":"10.1016\/j.mam.2016.05.004_bib1190","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1038\/gim.2015.10","article-title":"Lysosomal storage disease in the brain: mutations of the beta-mannosidase gene identified in autosomal dominant nystagmus","volume":"17","author":"Yu","year":"2015","journal-title":"Genet. Med"},{"issue":"2","key":"10.1016\/j.mam.2016.05.004_bib1195","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1002\/ijc.11536","article-title":"Suppression of 6A8 alpha-mannosidase gene expression reduced the potentiality of growth and metastasis of human nasopharyngeal carcinoma","volume":"108","author":"Yue","year":"2004","journal-title":"Int. J. Cancer"},{"issue":"3","key":"10.1016\/j.mam.2016.05.004_bib1200","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1093\/hmg\/ddi465","article-title":"Beta-mannosidosis mice: a model for the human lysosomal storage disease","volume":"15","author":"Zhu","year":"2006","journal-title":"Hum. Mol. Genet"}],"container-title":["Molecular Aspects of Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0098299716300103?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0098299716300103?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,6,3]],"date-time":"2025-06-03T19:51:16Z","timestamp":1748980276000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0098299716300103"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,10]]},"references-count":241,"alternative-id":["S0098299716300103"],"URL":"https:\/\/doi.org\/10.1016\/j.mam.2016.05.004","relation":{},"ISSN":["0098-2997"],"issn-type":[{"value":"0098-2997","type":"print"}],"subject":[],"published":{"date-parts":[[2016,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Catabolism of N-glycoproteins in mammalian cells: Molecular mechanisms and genetic disorders related to the processes","name":"articletitle","label":"Article Title"},{"value":"Molecular Aspects of Medicine","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.mam.2016.05.004","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2016 Published by Elsevier Ltd.","name":"copyright","label":"Copyright"}]}}