PCK1 ist ein Gen, das durch das Symbol PCK1 kodiert wird. Im Allgemeinen auch bezeichnet als: Phosphoenolpyruvate carboxykinase, cytosolic [GTP]; PEPCK-C; PEPCK1. PCK1 hat eine Masse von 69.2kDa, eine Aminosäurelänge von 622, und ist an Phosphoenolpyruvate carboxykinase deficiency, cytosolic beteiligt.
Wir bieten 6 antikörper gegen PCK1, aufgewachsen in Kaninchen, welche geeignet sind für WB, IHC and ELISA mit Proben abgeleitet von Human, Maus und Ratte.
Gen- und Proteininformationen
UniProt Zusammenfassung
Regulates cataplerosis and anaplerosis, the processes that control the levels of metabolic intermediates in the citric acid cycle. At low glucose levels, it catalyzes the cataplerotic 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. At high glucose levels, it catalyzes the anaplerotic conversion of phosphoenolpyruvate to oxaloacetate.
Entrez Zusammenfassung
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.
Gewebespezifität
Major sites of expression are liver, kidney and adipocytes.
Rolle bei Krankheiten
Phosphoenolpyruvate carboxykinase deficiency, cytosolic: An autosomal recessive metabolic disorder characterized by impaired gluconeogenesis, hypoglycemia, hypotonia, hepatomegaly, hepatic dysfunction, failure to thrive, lactic acidosis, and elevated tricarboxylic acid intermediates, particularly fumarate, in urine.
Sequenzähnlichkeiten
Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
Posttranslationale Modifikation
Acetylated. Lysine acetylation by p300/EP300 is increased on high glucose conditions (PubMed:20167786, PubMed:21726808, PubMed:30193097). Lysine acetylation promotes ubiquitination by UBR5 (PubMed:21726808). Acetylation is enhanced in the presence of BAG6. Deacetylated by SIRT2. Deacetylation of Lys-91 is carried out by SIRT1 and depends on PCK1 phosphorylation levels (PubMed:30193097).
