P35575.2
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
40,484 Da
NCBI Official Full Name
Glucose-6-phosphatase
NCBI Official Synonym Full Names
glucose-6-phosphatase, catalytic subunit
NCBI Protein Information
glucose-6-phosphatase; G6Pase; G-6-Pase; G6Pase-alpha; glucose-6-phosphatase alpha
UniProt Protein Name
Glucose-6-phosphatase
UniProt Synonym Protein Names
Glucose-6-phosphatase alpha
UniProt Synonym Gene Names
UniProt Entry Name
G6PC_HUMAN
NCBI Summary for G6PC
Glucose-6-phosphatase (G6Pase) is a multi-subunit integral membrane protein of the endoplasmic reticulum that is composed of a catalytic subunit and transporters for G6P, inorganic phosphate, and glucose. This gene (G6PC) is one of the three glucose-6-phosphatase catalytic-subunit-encoding genes in human: G6PC, G6PC2 and G6PC3. Glucose-6-phosphatase catalyzes the hydrolysis of D-glucose 6-phosphate to D-glucose and orthophosphate and is a key enzyme in glucose homeostasis, functioning in gluconeogenesis and glycogenolysis. Mutations in this gene cause glycogen storage disease type I (GSD1). This disease, also known as von Gierke disease, is a metabolic disorder characterized by severe hypoglycemia associated with the accumulation of glycogen and fat in the liver and kidneys.[provided by RefSeq, Feb 2011]
UniProt Comments for G6PC
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.
Protein type: EC 3.1.3.9; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - galactose; Endoplasmic reticulum; Carbohydrate Metabolism - starch and sucrose; Transporter; Transporter, SLC family; Membrane protein, multi-pass; Phosphatase (non-protein); Membrane protein, integral
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; phosphotransferase activity, alcohol group as acceptor; phosphate binding
Biological Process: response to food; glycogen metabolic process; steroid metabolic process; phosphorylated carbohydrate dephosphorylation; glycogen catabolic process; multicellular organism growth; glucose 6-phosphate metabolic process; pathogenesis; glucose transport; glucose homeostasis; gluconeogenesis; cholesterol homeostasis; urate metabolic process; triacylglycerol metabolic process; regulation of gene expression; glucose-6-phosphate transport; hexose transport; carbohydrate metabolic process; transmembrane transport
Disease: Glycogen Storage Disease Ia
Research Articles on G6PC
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Products associated with G6PC elisa kit
Pathways associated with G6PC elisa kit
Diseases associated with G6PC elisa kit
Organs/Tissues associated with G6PC elisa kit
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