NP_002582.3
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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
34,156 Da
NCBI Official Full Name
phosphoenolpyruvate carboxykinase, cytosolic
NCBI Official Synonym Full Names
phosphoenolpyruvate carboxykinase 1 (soluble)
NCBI Protein Information
phosphoenolpyruvate carboxykinase, cytosolic [GTP]; phosphoenolpyruvate carboxykinase, cytosolic [GTP]; PEP carboxykinase; phosphopyruvate carboxylase; phosphoenolpyruvate carboxylase
UniProt Protein Name
Phosphoenolpyruvate carboxykinase, cytosolic [GTP]
UniProt Synonym Gene Names
UniProt Entry Name
PCKGC_HUMAN
NCBI Summary for PCK1
This gene is a main control point for the regulation of gluconeogenesis. The cytosolic enzyme encoded by this gene, along with GTP, catalyzes the formation of phosphoenolpyruvate from oxaloacetate, with the release of carbon dioxide and GDP. The expression of this gene can be regulated by insulin, glucocorticoids, glucagon, cAMP, and diet. Defects in this gene are a cause of cytosolic phosphoenolpyruvate carboxykinase deficiency. A mitochondrial isozyme of the encoded protein also has been characterized. [provided by RefSeq, Jul 2008]
UniProt Comments for PCK1
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.
Protein type: Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Lyase; Kinase, other; EC 4.1.1.32
Chromosomal Location of Human Ortholog: 20q13.31
Cellular Component: cytoplasm; cytosol
Molecular Function: GDP binding; GTP binding; carboxylic acid binding; manganese ion binding; phosphoenolpyruvate carboxykinase (GTP) activity; magnesium ion binding
Biological Process: oxaloacetate metabolic process; carbohydrate metabolic process; glucose metabolic process; pathogenesis; response to activity; drug metabolic process; glucose homeostasis; internal protein amino acid acetylation; glycerol biosynthetic process from pyruvate; gluconeogenesis; response to insulin stimulus
Disease: Phosphoenolpyruvate Carboxykinase Deficiency, Cytosolic
Research Articles on PCK1
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Pathways associated with anti-PCK1 antibody
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