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Rabbit Anti-PCK1/FITC Conjugated antibody
background:
PCK1 is a main control point for the regulation of gluconeogenesis. This cytosolic enzyme, along with GTP, catalyzes the formation of phosphoenolpyruvate from oxaloacetate, with the release of carbon dioxide and GDP. The expression of the corresponding gene can be regulated by insulin, glucocorticoids, glucagon, cAMP, and diet. A mitochondrial isozyme has also been characterized.
Function:
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.
Subunit:
Monomer.
Subcellular Location:
Cytoplasm.
Tissue Specificity:
Major sites of expression are liver, kidney and adipocytes.
Post-translational modifications:
Acetylation is increased on addition of glucose and appears to regulate the protein stability.
DISEASE:
Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (SLCPEPCKD) [MIM:26336]. 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.
Similarity:
Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
Database links:
Entrez Gene: 5105 Human
Entrez Gene: 18534 Mouse
Entrez Gene: 362282 Rat
Omim: 614168 Human
SwissProt: P35558 Human
SwissProt: Q9Z2V4 Mouse
SwissProt: P07379 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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