Document Detail

Mannosidosis: separation and characterization of two acid alpha-mannosidase forms in mutant fibroblasts.
MedLine Citation:
PMID:  624273     Owner:  NLM     Status:  MEDLINE    
Two acidic forms of alpha-mannosidase activity, A and B, are separated and characterized in fibroblasts from controls and patients with mannosidosis. In normal cells, A and B differ in adsorption on DEAE-cellulose, electrophoretic mobility, stability at 70 degrees C and resistance to freezing at --20 degrees C. In 5 of 6 mutant cell lines, A and B both exhibit altered Km's for artificial substrates and decreased thermal stability. Zn2+ has no effect on A or B in mutant or normal cells. In contrast, Co2+ slightly inhibits B in normal cells but markedly enhances the activity of B in mutant cells. The mutation in mannosidosis clearly results in an alteration in the biochemical characteristics of both major acidic forms of alpha-mannosidase.
B K Burton; H L Nadler
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Enzyme     Volume:  23     ISSN:  0013-9432     ISO Abbreviation:  Enzyme     Publication Date:  1978  
Date Detail:
Created Date:  1978-04-17     Completed Date:  1978-04-17     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  1262265     Medline TA:  Enzyme     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  29-35     Citation Subset:  IM    
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MeSH Terms
Carbohydrate Metabolism, Inborn Errors / enzymology*
Cations, Divalent / pharmacology
Fibroblasts / enzymology
Glycosides / metabolism*
Hot Temperature
Isoelectric Point
Mannosidases / analysis*,  deficiency,  metabolism
Mannosides / metabolism*
Reg. No./Substance:
0/Cations, Divalent; 0/Glycosides; 0/Mannosides; EC 3.2.1.-/Mannosidases

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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