|Other Names||Pyruvate carboxylase, mitochondrial, Pyruvic carboxylase, PCB, PC|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.|
|Cellular Location||Mitochondrion matrix.|
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This gene encodes pyruvate carboxylase, which requiresbiotin and ATP to catalyse the carboxylation of pyruvate tooxaloacetate. The active enzyme is a homotetramer arranged in atetrahedron which is located exclusively in the mitochondrialmatrix. Pyruvate carboxylase is involved in gluconeogenesis,lipogenesis, insulin secretion and synthesis of theneurotransmitter glutamate. Mutations in this gene have beenassociated with pyruvate carboxylase deficiency. Alternativelyspliced transcript variants with different 5' UTRs, but encodingthe same protein, have been found for this gene. [provided byRefSeq].
MacDonald, M.J., et al. Diabetologia 52(6):1087-1091(2009)Monnot, S., et al. Hum. Mutat. 30(5):734-740(2009)Wang, D., et al. Mol. Genet. Metab. 95 (1-2), 31-38 (2008) :Vora, S., et al. Natl Med J India 21(3):116-119(2008)Xiang, S., et al. Nat. Struct. Mol. Biol. 15(3):295-302(2008)
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