Document Detail


Kinetic properties of beta-galactosidase in Morquio B disease.
MedLine Citation:
PMID:  6439230     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Some kinetic Properties of beta-galactosidase in crude extracts of cultured fibroblasts obtained from patients with Morquio B disease, GM1-gangliosidosis and from the controls were compared. The main defect in Morquio B seems to be the lowering of enzyme affinity to the substrates, while in the case of GM1-gangliosidosis of infantile type 1 a dramatic decrease of Vmax value concomitant with normal KM value was observed. Based on these observations possible interpretations of the clinical picture are discussed.
Authors:
B Czartoryska; D Gorska; T Abramowicz
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Acta anthropogenetica     Volume:  7     ISSN:  0258-0357     ISO Abbreviation:  Acta Anthropogenet     Publication Date:  1983  
Date Detail:
Created Date:  1985-02-11     Completed Date:  1985-02-11     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7801158     Medline TA:  Acta Anthropogenet     Country:  INDIA    
Other Details:
Languages:  eng     Pagination:  301-7     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Fibroblasts / enzymology
Galactosidases / metabolism*
Gangliosidoses / enzymology
Humans
Hydrogen-Ion Concentration
Kinetics
Mucopolysaccharidoses / enzymology*
beta-Galactosidase / antagonists & inhibitors,  metabolism*
Chemical
Reg. No./Substance:
EC 3.2.1.-/Galactosidases; EC 3.2.1.23/beta-Galactosidase

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


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