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Protein Page:

G6PC Hydrolyzes glucose-6-phosphate to glucose in the endoplasmic reticulum. Forms with the glucose-6-phosphate transporter (SLC37A4/G6PT) the complex responsible for glucose production through glycogenolysis and gluconeogenesis. Hence, it is the key enzyme in homeostatic regulation of blood glucose levels. Defects in G6PC are the cause of glycogen storage disease type 1A (GSD1A). A metabolic disorder characterized by impairment of terminal steps of glycogenolysis and gluconeogenesis. Patients manifest a wide range of clinical symptoms and biochemical abnormalities, including hypoglycemia, severe hepatomegaly due to excessive accumulation of glycogen, kidney enlargement, growth retardation, lactic acidemia, hyperlipidemia, and hyperuricemia. Belongs to the glucose-6-phosphatase family. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - galactose; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - starch and sucrose; EC; Endoplasmic reticulum; Membrane protein, integral; Membrane protein, multi-pass; Phosphatase (non-protein); Transporter; Transporter, SLC family
Chromosomal Location of Human Ortholog: 17q21.31
Cellular Component: endoplasmic reticulum membrane; integral to endoplasmic reticulum membrane; integral to membrane
Molecular Function: glucose-6-phosphatase activity; phosphate binding
Biological Process: gluconeogenesis; glucose 6-phosphate metabolic process; glucose homeostasis; glucose transport; glycogen metabolic process
Disease: Glycogen Storage Disease Ia
Reference #:  P35575 (UniProtKB)
Alt. Names/Synonyms: G-6-Pase; G6Pase; G6Pase-alpha; G6PC; G6PT; Glucose-6-phosphatase; Glucose-6-phosphatase alpha; glucose-6-phosphatase, catalytic subunit; GSD1; GSD1a; MGC163350
Gene Symbols: G6PC
Molecular weight: 40,484 Da
Basal Isoelectric point: 8.72  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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