Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1988-3-18
pubmed:abstractText
With pulse-chase study of 1-[14C]stearic acid-labeled cerebroside sulfate (14C-CS) and subsequent subcellular fractionation by Percoll gradient, the metabolism of CS and translocation of its metabolites in human skin fibroblasts from controls, metachromatic leukodystrophy (MLD), and globoid cell leukodystrophy (GLD) were studied. In control skin fibroblasts, CS was transported to lysosome and metabolized there to galactosylceramide (GalCer) and ceramide (Cer) within 1 h. During the chase period, radioactivity was increased at plasma membrane plus Golgi as phospholipids and no accumulation of GalCer or Cer was found in lysosome. In MLD fibroblasts, 95% of 14C-CS taken up was unhydrolyzed at 24 h-chase and accumulated at not only lysosome but also plasma membrane. In GLD fibroblasts, GalCer was accumulated throughout the subcellular fractions and more accumulated mainly at plasma membrane plus Golgi with longer pulse. This translocation of lipid from lysosome seems to have considerable function, even in lipidosis, which may result in an imbalance of the sphingolipid pattern on the cell surface and these changes might be one of causes of neuronal dysfunction in sphingolipidosis.
pubmed:commentsCorrections
http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-14907713, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-185697, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-25315, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-2931941, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-2983023, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-3919000, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-3922757, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-3934152, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-3965473, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-3995063, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-415798, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-5047687, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-6107060, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-6126152, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-6202736, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-6220018, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-6224417, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-6719111, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-6806321, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-7023369, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-7061463, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-7159437, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-731265, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-7354254, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-824017, http://linkedlifedata.com/resource/pubmed/commentcorrection/3339122-901761
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0021-9738
pubmed:author
pubmed:issnType
Print
pubmed:volume
81
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
310-7
pubmed:dateRevised
2009-11-18
pubmed:meshHeading
pubmed:year
1988
pubmed:articleTitle
Metabolism of cerebroside sulfate and subcellular distribution of its metabolites in cultured skin fibroblasts from controls, metachromatic leukodystrophy, and globoid cell leukodystrophy.
pubmed:affiliation
Department of Pediatrics, Osaka University Medical School, Japan.
pubmed:publicationType
Journal Article, In Vitro, Research Support, Non-U.S. Gov't