Document Detail

Liver microsomal transport of glucose-6-phosphate, glucose, and phosphate in type 1 glycogen storage disease.
MedLine Citation:
PMID:  9435446     Owner:  NLM     Status:  MEDLINE    
The transport of glucose-6-phosphate (G6P), glucose, and orthophosphate into liver microsomes, isolated from six patients with various subtypes of type 1 glycogen storage disease (GSD), was measured using a light-scattering method. We found that G6P, glucose, and phosphate could all cross the microsomal membrane, in four cases of type 1a GSD. In contrast, liver microsomal transport of G6P and phosphate was deficient in the GSD 1b and 1c patients, respectively. These results support the involvement of multiple proteins (and genes) in GSD type 1. The results obtained with the light-scattering method are in accordance with conventional kinetic analysis of the microsomal glucose-6-phosphatase system. Therefore, this technique could be used to directly diagnose type 1b and 1c GSD.
P Marcolongo; G Bánhegyi; A Benedetti; C J Hinds; A Burchell
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of clinical endocrinology and metabolism     Volume:  83     ISSN:  0021-972X     ISO Abbreviation:  J. Clin. Endocrinol. Metab.     Publication Date:  1998 Jan 
Date Detail:
Created Date:  1998-02-09     Completed Date:  1998-02-09     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375362     Medline TA:  J Clin Endocrinol Metab     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  224-9     Citation Subset:  AIM; IM    
Istituto di Patologia Generale, Univesità di Siena, Italy.
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MeSH Terms
Glucose / metabolism*
Glucose-6-Phosphate / metabolism*
Glycogen Storage Disease Type I / metabolism*
Microsomes, Liver / metabolism*
Phosphates / metabolism
Reference Values
Scattering, Radiation
Reg. No./Substance:
0/Phosphates; 50-99-7/Glucose; 56-73-5/Glucose-6-Phosphate

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