{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T13:44:49Z","timestamp":1791812689707,"version":"4.3.4"},"reference-count":46,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2007,4]]},"DOI":"10.1016\/j.ymgme.2006.11.006","type":"journal-article","created":{"date-parts":[[2006,12,21]],"date-time":"2006-12-21T13:05:03Z","timestamp":1166706303000},"page":"393-401","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":93,"title":["Lysosomal accumulation of SCMAS (subunit c of mitochondrial ATP synthase) in neurons of the mouse model of mucopolysaccharidosis III B"],"prefix":"10.1016","volume":"90","author":[{"given":"Sergey","family":"Ryazantsev","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei-Hong","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hui-Zhi","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth F.","family":"Neufeld","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhiro","family":"Ohmi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgme.2006.11.006_bib1","series-title":"The Metabolic and Molecular Bases of Inherited Disease","first-page":"3421","article-title":"The mucopolysaccharidoses","author":"Neufeld","year":"2001"},{"key":"10.1016\/j.ymgme.2006.11.006_bib2","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1002\/humu.1189","article-title":"Molecular genetics of mucopolysaccharidosis type IIIA and IIIB: diagnostic, clinical, and biological implications","volume":"18","author":"Yogalingam","year":"2001","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.ymgme.2006.11.006_bib3","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1136\/adc.69.3.403","article-title":"Management of mucopolysaccharidosis type III","volume":"69","author":"Cleary","year":"1993","journal-title":"Arch. Dis. Child."},{"key":"10.1016\/j.ymgme.2006.11.006_bib4","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1006\/geno.2001.6552","article-title":"Molecular basis of mucopolysaccharidosis type IIIB in emu (Dromaius novaehollandiae): an avian model of Sanfilippo syndrome type B","volume":"74","author":"Aronovich","year":"2001","journal-title":"Genomics"},{"key":"10.1016\/j.ymgme.2006.11.006_bib5","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1023\/A:1025177411938","article-title":"A model of mucopolysaccharidosis IIIB (Sanfilippo syndrome type IIIB): N-acetyl-alpha-d-glucosaminidase deficiency in Schipperke dogs","volume":"26","author":"Ellinwood","year":"2003","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2006.11.006_bib6","doi-asserted-by":"crossref","first-page":"14505","DOI":"10.1073\/pnas.96.25.14505","article-title":"Mouse model of Sanfilippo syndrome type B produced by targeted disruption of the gene encoding alpha-N-acetylglucosaminidase","volume":"96","author":"Li","year":"1999","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.ymgme.2006.11.006_bib7","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1001\/archneur.1980.00500590069012","article-title":"Pathologic findings in mucopolysaccharidosis type IIIB (Sanfilippo\u2019s sydnrome B)","volume":"37","author":"Hadfield","year":"1980","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib8","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/j.semcdb.2004.03.002","article-title":"Secondary accumulation of gangliosides in lysosomal storage disorders","volume":"15","author":"Walkley","year":"2004","journal-title":"Semin. Cell Dev. Biol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib9","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1002\/cne.20355","article-title":"Differential subcellular localization of cholesterol, gangliosides, and glycosaminoglycans in murine models of mucopolysaccharide storage disorders","volume":"480","author":"McGlynn","year":"2004","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib10","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1002\/ana.10373","article-title":"Selective neurodegeneration in murine mucopolysaccharidosis VII is progressive and reversible","volume":"52","author":"Heuer","year":"2002","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib11","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1016\/j.ymgme.2004.05.005","article-title":"Intracerebral injection of sulfamidase delays neuropathology in murine MPS-IIIA","volume":"82","author":"Savas","year":"2004","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2006.11.006_bib12","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1007\/s004010050629","article-title":"Follow-up study of subunit c of mitochondrial ATP synthase (SCMAS) in Batten disease and in unrelated lysosomal disorders","volume":"93","author":"Elleder","year":"1997","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.ymgme.2006.11.006_bib13","doi-asserted-by":"crossref","first-page":"39045","DOI":"10.1074\/jbc.X200001200","article-title":"A research journey with ATP synthase","volume":"277","author":"Boyer","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2006.11.006_bib14","doi-asserted-by":"crossref","first-page":"5736","DOI":"10.1016\/S0021-9258(18)83610-9","article-title":"Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence","volume":"264","author":"Palmer","year":"1989","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2006.11.006_bib15","doi-asserted-by":"crossref","first-page":"1802","DOI":"10.1126\/science.277.5333.1802","article-title":"Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis","volume":"277","author":"Sleat","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.ymgme.2006.11.006_bib16","doi-asserted-by":"crossref","first-page":"2573","DOI":"10.1046\/j.1471-4159.1999.0722573.x","article-title":"A lysosomal proteinase, the late infantile neuronal ceroid lipofuscinosis gene (CLN2) product, is essential for degradation of a hydrophobic protein, the subunit c of ATP synthase","volume":"72","author":"Ezaki","year":"1999","journal-title":"J. Neurochem."},{"key":"10.1016\/j.ymgme.2006.11.006_bib17","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1023\/A:1005665732189","article-title":"Tripeptidyl-peptidase I deficiency in classical late-infantile neuronal ceroid lipofuscinosis brain tissue. Evidence for defective peptidase rather than proteinase activity","volume":"23","author":"Warburton","year":"2000","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2006.11.006_bib18","doi-asserted-by":"crossref","first-page":"6898","DOI":"10.1523\/JNEUROSCI.20-18-06898.2000","article-title":"Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons","volume":"20","author":"Koike","year":"2000","journal-title":"J. Neurosci."},{"key":"10.1016\/j.ymgme.2006.11.006_bib19","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1038\/sj.emboj.7600576","article-title":"Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration","volume":"24","author":"Kasper","year":"2005","journal-title":"EMBO J."},{"key":"10.1016\/j.ymgme.2006.11.006_bib20","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/j.ymgme.2004.06.004","article-title":"Retrovirally transduced bone marrow has a therapeutic effect on brain in the mouse model of mucopolysaccharidosis IIIB","volume":"82","author":"Zheng","year":"2004","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2006.11.006_bib21","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/0167-4781(93)90249-D","article-title":"Molecular cloning and sequence of two cDNAs for human subunit c of H(+)-ATP synthase in mitochondria","volume":"1173","author":"Higuti","year":"1993","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgme.2006.11.006_bib22","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1002\/ajmg.1320570230","article-title":"Batten disease and the ATP synthase subunit c turnover pathway: raising antibodies to subunit c","volume":"57","author":"Palmer","year":"1995","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.ymgme.2006.11.006_bib23","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/S0167-4838(98)00012-0","article-title":"Purification and characterisation of a tripeptidyl aminopeptidase I from rat spleen","volume":"1384","author":"Vines","year":"1998","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgme.2006.11.006_bib24","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1016\/0304-4165(96)00013-X","article-title":"Dipeptidyl peptidase II from porcine seminal plasma: purification, characterization, and its homology to granzymes, cytotoxic cell proteinases (CCP 1-4)","volume":"1290","author":"Huang","year":"1996","journal-title":"Biochim. Biophys. Acta"},{"issue":"Pt C","key":"10.1016\/j.ymgme.2006.11.006_bib25","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1016\/S0076-6879(81)80043-2","article-title":"Cathepsin B, Cathepsin H, and cathepsin L","volume":"80","author":"Barrett","year":"1981","journal-title":"Methods Enzymol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib26","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1016\/S0305-0491(98)00030-3","article-title":"Purification and properties of embryonic cysteine proteinase which participates in yolk-lysis of Xenopus laevis","volume":"119","author":"Yoshizaki","year":"1998","journal-title":"Comp. Biochem. Physiol. B Biochem. Mol. Biol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib27","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/S0305-0491(96)00249-0","article-title":"Substrate specificity of carp Cyprinus carpio cathepsin H with methylcoumarylamide substrates","volume":"116","author":"Aranishi","year":"1997","journal-title":"Comp. Biochem. Physiol. B Biochem. Mol. Biol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib28","doi-asserted-by":"crossref","first-page":"1137","DOI":"10.1093\/oxfordjournals.jbchem.a022396","article-title":"Characterization of new fluorogenic substrates for the rapid and sensitive assay of cathepsin E and cathepsin D","volume":"125","author":"Yasuda","year":"1999","journal-title":"J. Biochem. (Tokyo)"},{"key":"10.1016\/j.ymgme.2006.11.006_bib29","series-title":"Theory and Practice of Histotechnology","author":"Sheehan","year":"1980"},{"key":"10.1016\/j.ymgme.2006.11.006_bib30","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1093\/jnen\/60.1.49","article-title":"Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations","volume":"60","author":"Zervas","year":"2001","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib31","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1083\/jcb.57.2.551","article-title":"A technique for ultracryotomy of cell suspensions and tissues","volume":"57","author":"Tokuyasu","year":"1973","journal-title":"J. Cell. Biol."},{"issue":"Pt 2","key":"10.1016\/j.ymgme.2006.11.006_bib32","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1111\/j.1365-2818.1986.tb02772.x","article-title":"Application of cryoultramicrotomy to immunocytochemistry","volume":"143","author":"Tokuyasu","year":"1986","journal-title":"J. Microsc."},{"key":"10.1016\/j.ymgme.2006.11.006_bib33","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1007\/BF01007491","article-title":"Use of poly(vinylpyrrolidone) and poly(vinyl alcohol) for cryoultramicrotomy","volume":"21","author":"Tokuyasu","year":"1989","journal-title":"Histochem. J."},{"key":"10.1016\/j.ymgme.2006.11.006_bib34","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1007\/BF02473201","article-title":"Improving structural integrity of cryosections for immunogold labeling","volume":"106","author":"Liou","year":"1996","journal-title":"Histochem. Cell Biol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib35","doi-asserted-by":"crossref","first-page":"1902","DOI":"10.1073\/pnas.252784899","article-title":"Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB","volume":"100","author":"Ohmi","year":"2003","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.ymgme.2006.11.006_bib36","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1097\/00005072-196504000-00010","article-title":"Electron microscopy of two cerebral biopsies in Gargoylism","volume":"24","author":"Aleu","year":"1965","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib37","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1002\/ana.410020214","article-title":"Neuropathology of Sanfilippo syndrome","volume":"2","author":"Ghatak","year":"1977","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib38","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1177\/36.11.3171166","article-title":"Autophagy of mitochondria in rat liver assessed by immunogold procedures","volume":"36","author":"Knecht","year":"1988","journal-title":"J. Histoch. Cytochem."},{"key":"10.1016\/j.ymgme.2006.11.006_bib39","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1038\/nature04723","article-title":"Loss of autophagy in the central nervous system causes neurodegeneration in mice","volume":"441","author":"Komatsu","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.ymgme.2006.11.006_bib40","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1038\/nature04724","article-title":"Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice","volume":"441","author":"Hara","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.ymgme.2006.11.006_bib41","doi-asserted-by":"crossref","first-page":"1431","DOI":"10.1515\/bchm2.1977.358.2.1431","article-title":"Characterization of glycosaminoglycans stored in mucopolysaccharidosis III A: evidence for a generally occuring degradation of heparan sulfate by endoglycosidases","volume":"358","author":"Kindler","year":"1977","journal-title":"Hoppe Seylers Z. Physiol. Chem."},{"key":"10.1016\/j.ymgme.2006.11.006_bib42","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/j.ymgme.2005.09.026","article-title":"Validation of a heparan sulfate-derived disaccharide as a marker of accumulation in murine mucopolysaccharidosis type IIIA","volume":"87","author":"King","year":"2006","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2006.11.006_bib43","doi-asserted-by":"crossref","first-page":"4500","DOI":"10.1091\/mbc.e04-05-0432","article-title":"Elevated endosomal cholesterol levels in Niemann-Pick cells inhibit rab4 and perturb membrane recycling","volume":"15","author":"Choudhury","year":"2004","journal-title":"Mol. Biol. Cell."},{"key":"10.1016\/j.ymgme.2006.11.006_bib44","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1016\/S0962-8924(02)02399-1","article-title":"Endocytosis and sorting of glycosphingolipids in sphingolipid storage disease","volume":"12","author":"Marks","year":"2002","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib45","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/S0955-0674(03)00010-3","article-title":"Ubiquitin: not just for proteasomes anymore","volume":"15","author":"Aguilar","year":"2003","journal-title":"Curr. Opin. Cell Biol."},{"key":"10.1016\/j.ymgme.2006.11.006_bib46","series-title":"The Mouse Brain In Stereotactic Coordinates","author":"Paxinos","year":"2001"}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719206003647?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719206003647?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,8,16]],"date-time":"2024-08-16T19:02:10Z","timestamp":1723834930000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719206003647"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,4]]},"references-count":46,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2007,4]]}},"alternative-id":["S1096719206003647"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2006.11.006","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2007,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Lysosomal accumulation of SCMAS (subunit c of mitochondrial ATP synthase) in neurons of the mouse model of mucopolysaccharidosis III B","name":"articletitle","label":"Article Title"},{"value":"Molecular Genetics and Metabolism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymgme.2006.11.006","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2006 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}