Description
Product Characteristics:
Glucose-6-phosphatase (G6Pase), is a multicomponent enzyme system that hydrolyzes glucose-6-phosphate (G6P) in the final step of gluconeogenesis and gluconeolysis. G6Pase localizes to the endoplasmic reticulum, and while liver, kidney, and intestine are the only tissues that express the first identified isoform, G6Pase-Alpha, a second form, designated G6Pase-Beta, contributes to blood glucose homeostasis in a wider range of tissues. G6Pase-Beta, also known as SCN4, UGRP or G6PC3 (glucose 6 phosphatase, catalytic, 3), is a 346 amino acid endoplasmic reticulum multi-pass membrane protein that is involved in carbohydrate biosynthesis and the gluconeogenesis pathway. Inhibited by vanadate, G6Pase-Beta hydrolyzes GP6 to glucose in the endoplasmic reticulum. Due to its necessary involvement in normal glucose metabolism, G6Pase-Beta may play an integral role in diabetes and glycogen storage diseases (GSDs).
Subcellular location: Cytoplasm, Cell membrane
Synonyms: G-6-Pase 3, G6Pase 3, G6Pase-beta, Glucose 6 phosphatase, catalytic, 3, Glucose-6-phosphatase 3, Glucose-6-phosphatase beta, SCN4, Ubiquitous glucose-6-phosphatase catalytic subunit-related protein, UGRP,
Target Information: This gene encodes the catalytic subunit of glucose-6-phosphatase (G6Pase). G6Pase is located in the endoplasmic reticulum (ER) and catalyzes the hydrolysis of glucose-6-phosphate to glucose and phosphate in the last step of the gluconeogenic and glycogenolytic pathways. Mutations in this gene result in autosomal recessive severe congenital neutropenia. Multiple transcript variants have been found for this gene, only one of which is expected to express a protein.[provided by RefSeq, Sep 2009]