pyrophosphate translocase
a component of the glucose-6-phosphatase (G6Pase, EC 3.1.3.9) enzyme complex; facilitates the transfer of pyrophosphate between the cell cytosol and the lumen of the endoplasmic reticulum where the G6Pase enzyme is situated
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Related Diseases
1. | Glycogen Storage Disease (Glycogenosis)
06/01/2006
- " Glycogen storage diseases (GSDs) are characterized by abnormal inherited glycogen metabolism in the liver, muscle, and brain and divided into types 0 to X. GSD type I, glucose 6-phosphatase system, has types Ia, Ib, Ic, and Id, glucose 6-phosphatase, glucose 6-phosphate translocase, pyrophosphate translocase, and glucose translocase deficiencies, respectively. " 06/01/1989
- " 1. Type 1b and type 1c glycogen-storage disease are caused respectively by deficiencies of the glucose-6-phosphate translocase and the phosphate/pyrophosphate translocase of the human hepatic microsomal glucose-6-phosphatase system. "
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2. | Hepatomegaly
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