NP_000142.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
20,209 Da
NCBI Official Full Name
glucose-6-phosphatase isoform 1
NCBI Official Synonym Full Names
glucose-6-phosphatase catalytic subunit
NCBI Official Synonym Symbols
G6PT; GSD1; G6PC1; GSD1a; G6Pase [Similar Products]
NCBI Protein Information
glucose-6-phosphatase
UniProt Protein Name
Glucose-6-phosphatase
UniProt Synonym Protein Names
Glucose-6-phosphatase alpha; G6Pase-alpha
UniProt Synonym Gene Names
NCBI Summary for G-6-Pase
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 G-6-Pase
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: Carbohydrate Metabolism - galactose; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - starch and sucrose; EC 3.1.3.9; 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 component of endoplasmic reticulum membrane; integral component of membrane
Molecular Function: glucose-6-phosphatase activity; phosphate binding; phosphotransferase activity, alcohol group as acceptor
Biological Process: cholesterol homeostasis; gluconeogenesis; glucose 6-phosphate metabolic process; glucose homeostasis; glucose-6-phosphate transport; glycogen catabolic process; glycogen metabolic process; multicellular organism growth; phosphorylated carbohydrate dephosphorylation; regulation of gene expression; response to food; steroid metabolic process; triacylglycerol metabolic process; urate metabolic process
Disease: Glycogen Storage Disease Ia
Research Articles on G-6-Pase
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Pathways associated with G-6-Pase elisa kit
Diseases associated with G-6-Pase elisa kit
Organs/Tissues associated with G-6-Pase elisa kit
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