|Other Names||2-oxoglutarate dehydrogenase, mitochondrial, 2-oxoglutarate dehydrogenase complex component E1, OGDC-E1, Alpha-ketoglutarate dehydrogenase, OGDH|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP13442b was selected from the C-term region of OGDH. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|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||The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2- oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).|
|Cellular Location||Mitochondrion matrix.|
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Provided below are standard protocols that you may find useful for product applications.
This gene encodes one subunit of the 2-oxoglutaratedehydrogenase complex. This complex catalyzes the overallconversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoAand CO(2) during the Krebs cycle. The protein is located in themitochondrial matrix and uses thiamine pyrophosphate as a cofactor.A congenital deficiency in 2-oxoglutarate dehydrogenase activity isbelieved to lead to hypotonia, metabolic acidosis, andhyperlactatemia. Alternative splicing results in multipletranscript variants encoding distinct isoforms.
van Bever, Y., et al. Am. J. Med. Genet. A 143(7):763-767(2007)Shi, Q., et al. J. Biol. Chem. 280(12):10888-10896(2005)Habelhah, H., et al. J. Biol. Chem. 279(51):53782-53788(2004)McCartney, R.G., et al. J. Biol. Chem. 273(37):24158-24164(1998)Koike, K. Gene 159(2):261-266(1995)
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