Intestinal Glucose-dependent Expression of Glucose-6-phosphatase

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Glucose-6-phosphatase deficiency

Glucose-6-phosphatase deficiency (G6P deficiency), or glycogen storage disease type I (GSDI), is a group of inherited metabolic diseases, including types Ia and Ib, characterized by poor tolerance to fasting, growth retardation and hepatomegaly resulting from accumulation of glycogen and fat in the liver. Prevalence is unknown and annual incidence is around 1/100,000 births. GSDIa is the more f...

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1. The existence of specific glucose-6-phosphatase activity in human intestinal mucosa has been somewhat controversial. 2. We have demonstrated the presence of low levels of specific glucose-6-phosphatase activity in normal human adult intestinal mucosa. Activity was found in oesophagus, stomach, duodenum and colon. 3. Immunoblot analysis using antibodies monospecific for the 36.5 kDa liver glu...

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The glucose-6-phosphatase system.

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Liver Microsomal Glucose 6 - Phosphatase , Inorganic Pyrophosphatase , and Pyrophosphate - Glucose

Recently, we (1) described experiments which strongly supported the common identity of glucose 6-phosphatase,’ inorganic pyrophosphatase, pyrophosphate-glucose phosphotransferase, and mannose B-phosphate-glucose phosphotransferase activities present in a preparation obtained by ammonium sulfate fractionation of deoxycholate-dispersed rat liver microsomes. Previously, Segal, Washko, and Lee (2, ...

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ژورنال

عنوان ژورنال: Journal of Biological Chemistry

سال: 2005

ISSN: 0021-9258

DOI: 10.1074/jbc.m502192200