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Protein Page:
PCK1 (human)

PCK1 Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle. Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (C-PEPCKD). A metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait. Belongs to the phosphoenolpyruvate carboxykinase [GTP] family. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - pyruvate; EC; Kinase, other; Lyase
Chromosomal Location of Human Ortholog: 20q13.31
Cellular Component: cytoplasm; cytosol
Molecular Function: carboxylic acid binding; GTP binding; magnesium ion binding; manganese ion binding; phosphoenolpyruvate carboxykinase (GTP) activity
Biological Process: gluconeogenesis; glucose homeostasis; glucose metabolic process; glycerol biosynthetic process from pyruvate; internal protein amino acid acetylation; response to insulin stimulus
Disease: Phosphoenolpyruvate Carboxykinase Deficiency, Cytosolic
Reference #:  P35558 (UniProtKB)
Alt. Names/Synonyms: MGC22652; PCK1; PCKGC; PEP carboxykinase; PEPCK-C; PEPCK1; PEPCKC; phosphoenolpyruvate carboxykinase 1 (soluble); phosphoenolpyruvate carboxykinase, cytosolic; Phosphoenolpyruvate carboxykinase, cytosolic [GTP]; Phosphoenolpyruvate carboxylase; phosphopyruvate carboxylase
Gene Symbols: PCK1
Molecular weight: 69,195 Da
Basal Isoelectric point: 5.8  Predict pI for various phosphorylation states
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