Liver microsomal transport of glucose-6-phosphate, glucose, and phosphate in type 1 glycogen storage disease.
J Clin Endocrinol Metab
; 83(1): 224-9, 1998 Jan.
Article
em En
| MEDLINE
| ID: mdl-9435446
ABSTRACT
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Microssomos Hepáticos
/
Doença de Depósito de Glicogênio Tipo I
/
Glucose-6-Fosfato
/
Glucose
Limite:
Adult
/
Animals
/
Humans
Idioma:
En
Revista:
J Clin Endocrinol Metab
Ano de publicação:
1998
Tipo de documento:
Article
País de afiliação:
Itália