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Protein Page:
G6PC (human)

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: Transporter; Membrane protein, multi-pass; Carbohydrate Metabolism - glycolysis and gluconeogenesis; EC; Carbohydrate Metabolism - starch and sucrose; Endoplasmic reticulum; Transporter, SLC family; Carbohydrate Metabolism - galactose; Membrane protein, integral; Phosphatase (non-protein)
Chromosomal Location of Human Ortholog: 17q21
Cellular Component: endoplasmic reticulum membrane; integral to membrane; integral to endoplasmic reticulum membrane
Molecular Function: glucose-6-phosphatase activity; phosphate binding; phosphotransferase activity, alcohol group as acceptor
Biological Process: response to food; steroid metabolic process; glycogen metabolic process; phosphorylated carbohydrate dephosphorylation; glycogen catabolic process; multicellular organism growth; glucose 6-phosphate metabolic process; pathogenesis; glucose transport; glucose homeostasis; gluconeogenesis; cholesterol homeostasis; triacylglycerol metabolic process; urate metabolic process; glucose-6-phosphate transport; regulation of gene expression; hexose transport; carbohydrate metabolic process; transmembrane transport
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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Modification Sites and Domains  

Modification Sites in Parent Protein, Orthologs, and Isoforms  

Show Multiple Sequence Alignment


SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.



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