| Deficient phosphorylation of mannose residues of mannan in fibroblasts of patients with mucolipidoses II and III. | |
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MedLine Citation:
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PMID: 7305949 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Incorporation of 32P from [gamma 32P]ATP into mannan could not be detected in homogenates of cultivated skin fibroblasts from patients with mucolipidosis II, and accounted for only up to 10% of normal control activity in cell lysates from patients with mucolipidosis III. Parents of patients with mucolipidosis II demonstrated 60-70% of normal control activity. On high-voltage electrophoresis, the hydrolysed mannan from reactions performed with normal cells, over the pH range 5.5-7.5, yielded a radioactive band migrating with the same mobility as mannose 6-phosphate, whereas no such product could be demonstrated in fibroblasts of patients with mucolipidosis II. |
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Authors:
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Y Ben-Yoseph; L C Hahn; C L DeFranco; H L Nadler |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: The Biochemical journal Volume: 193 ISSN: 0264-6021 ISO Abbreviation: Biochem. J. Publication Date: 1981 Feb |
Date Detail:
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Created Date: 1982-01-28 Completed Date: 1982-01-28 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 2984726R Medline TA: Biochem J Country: ENGLAND |
Other Details:
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Languages: eng Pagination: 651-4 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Cells, Cultured Electrophoresis, Paper Fibroblasts / metabolism Glucose / metabolism Humans Mannans / metabolism* Mannose / metabolism* Mucolipidoses / metabolism* Phosphorylation Polysaccharides / metabolism* Skin / metabolism |
| Chemical | |
Reg. No./Substance:
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0/Mannans; 0/Polysaccharides; 31103-86-3/Mannose; 50-99-7/Glucose |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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