PEPCK-C Antibody – Rabbit Polyclonal

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; Kinase, other; Carbohydrate Metabolism - citrate (TCA) cycle; Lyase; EC 4.1.1.32; Carbohydrate Metabolism - glycolysis and gluconeogenesis

Cellular Component: cytoplasm; cytosol

Molecular Function: GDP binding; GTP binding; purine nucleotide binding; carboxylic acid binding; manganese ion binding; phosphoenolpyruvate carboxykinase (GTP) activity; metal ion binding; nucleotide binding; lyase activity; magnesium ion binding; carboxy-lyase activity; phosphoenolpyruvate carboxykinase activity

Biological Process: cellular response to potassium ion starvation; oxaloacetate metabolic process; glucose metabolic process; lipid metabolic process; glucose homeostasis; glycerol biosynthetic process from pyruvate; internal protein amino acid acetylation; gluconeogenesis; response to insulin stimulus