{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T20:27:26Z","timestamp":1786998446835,"version":"build-2736575974"},"reference-count":18,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2014,4,1]],"date-time":"2014-04-01T00:00:00Z","timestamp":1396310400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"name":"Margaret Enoch Fund"},{"name":"NIH","award":["NS055195"],"award-info":[{"award-number":["NS055195"]}]},{"name":"Boston College Research Expense Fund"}],"content-domain":{"domain":["mgmjournal.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2014,4]]},"DOI":"10.1016\/j.ymgme.2014.02.002","type":"journal-article","created":{"date-parts":[[2014,2,13]],"date-time":"2014-02-13T00:31:47Z","timestamp":1392251507000},"page":"513-521","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":16,"title":["GM1-gangliosidosis in American black bears: Clinical, pathological, biochemical and molecular genetic characterization"],"prefix":"10.1016","volume":"111","author":[{"given":"Sureshkumar","family":"Muthupalani","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola A.","family":"Torres","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Betty C.","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bai Jin","family":"Zeng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel","family":"Eaton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ildiko","family":"Erdelyi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebecca","family":"Ducore","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajanikarath","family":"Maganti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Keating","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bain J.","family":"Perry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florina S.","family":"Tseng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Waliszewski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Pokras","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Causey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita","family":"Seger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"March","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Tidwell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf","family":"Pfannl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Seyfried","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edwin H.","family":"Kolodny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Alroy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgme.2014.02.002_bb0005","series-title":"The Metabolic & Molecular Bases of Inherited Disease","first-page":"3775","article-title":"\u03b2-Galactosidase deficiency (\u03b2-galactosidosis): GM1 gangliosidosis and Morquio B disease","author":"Suzuki","year":"2001"},{"key":"10.1016\/j.ymgme.2014.02.002_bb0010","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1177\/104063870001200601","article-title":"Morphological, biochemical and molecular biology approaches for the diagnosis of lysosomal storage diseases","volume":"12","author":"Warren","year":"2000","journal-title":"J. Vet. Diagn. Invest."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0015","doi-asserted-by":"crossref","first-page":"292","DOI":"10.2307\/1591870","article-title":"Gangliosidosis in emus (Dromaius novaehollandiae)","volume":"39","author":"Bermudez","year":"1995","journal-title":"Avian Dis."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0020","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/S0021-9258(18)64849-5","article-title":"A simple method for the isolation and purification of total lipides from animal tissues","volume":"226","author":"Folch","year":"1957","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0025","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1042\/bj0810384","article-title":"Methods for the quantitative estimation of N-acetylneuraminic acid and their application to hydrolysates of sialomucoids","volume":"81","author":"Aminoff","year":"1961","journal-title":"Biochem. J."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0030","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1023\/B:BIGI.0000034429.55418.71","article-title":"Inheritance of lysosomal acid beta-galactosidase activity and gangliosides in crosses of DBA\/2J and knockout mice","volume":"42","author":"Hauser","year":"2004","journal-title":"Biochem. Genet."},{"issue":"2009","key":"10.1016\/j.ymgme.2014.02.002_bb0035","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1007\/s11745-008-3268-0","article-title":"Comparative analysis of brain lipids in mice, cats, and humans with Sandhoff disease","volume":"44","author":"Baek","year":"2009","journal-title":"Lipids"},{"key":"10.1016\/j.ymgme.2014.02.002_bb0040","doi-asserted-by":"crossref","first-page":"1243","DOI":"10.1016\/S0022-2275(20)37906-2","article-title":"Analysis of brain lipids by high performance thin-layer chromatography and densitometry","volume":"24","author":"Macula","year":"1983","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0045","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1016\/0006-3002(57)90254-8","article-title":"Quantitative estimation of sialic acids. II. A colorimetric resorcinol-hydrochloric acid method","volume":"24","author":"Svennerholm","year":"1957","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgme.2014.02.002_bb0050","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1111\/j.1471-4159.1978.tb12428.x","article-title":"Cerebral, cerebellar, and brain stem gangliosides in mice susceptible to audiogenic seizures","volume":"31","author":"Seyfried","year":"1978","journal-title":"J. Neurochem."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0055","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1046\/j.1471-4159.2004.02381.x","article-title":"N-butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis","volume":"89","author":"Kasperzyk","year":"2004","journal-title":"J. Neurochem."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0060","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1126\/science.3925555","article-title":"Neurovisceral and skeletal GM1-gangliosidosis in dogs with beta-galactosidase deficiency","volume":"229","author":"Alroy","year":"1985","journal-title":"Science"},{"key":"10.1016\/j.ymgme.2014.02.002_bb0065","first-page":"675","article-title":"Canine GM1-gangliosidosis. A clinical, morphologic, histochemical, and biochemical comparison of two different models","volume":"140","author":"Alroy","year":"1992","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0070","doi-asserted-by":"crossref","first-page":"1294","DOI":"10.1111\/j.1471-4159.2007.04525.x","article-title":"Neurochemical, morphological and neurophysiological abnormalities in retinas of Sandhoff and GM1 gangliosidosis mice","volume":"101","author":"Denny","year":"2007","journal-title":"J. Neurochem."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0075","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1016\/0887-8994(92)90361-2","article-title":"Dysmyelinogenesis in animal model of GM1 gangliosidosis","volume":"8","author":"Kaye","year":"1992","journal-title":"Pediatr. Neurol."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0080","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1007\/BF00192635","article-title":"Retarded bone formation in GM1-gangliosidosis: a study of the infantile form and comparison with two canine models","volume":"426","author":"Alroy","year":"1995","journal-title":"Virchows Arch."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0085","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1007\/BF02899193","article-title":"Lectin histochemistry and ultrastructure of feline kidneys from six different storage diseases","volume":"54","author":"Castagnaro","year":"1987","journal-title":"Virchows Arch. B Cell Pathol. Incl. Mol. Pathol."},{"key":"10.1016\/j.ymgme.2014.02.002_bb0090","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1023\/A:1005630013448","article-title":"Isolation and characterization of the normal canine \u03b2-galactosidase gene and its mutation in a dog model of GM1-gangliosidosis","volume":"23","author":"Wang","year":"2000","journal-title":"J. Inherit. Metab. Dis."}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719214000651?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719214000651?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,3,26]],"date-time":"2022-03-26T09:35:58Z","timestamp":1648287358000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719214000651"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,4]]},"references-count":18,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2014,4]]}},"alternative-id":["S1096719214000651"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2014.02.002","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2014,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"GM1-gangliosidosis in American black bears: Clinical, pathological, biochemical and molecular genetic characterization","name":"articletitle","label":"Article Title"},{"value":"Molecular Genetics and Metabolism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymgme.2014.02.002","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2014 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}