|Other Names||Isovaleryl-CoA dehydrogenase, mitochondrial, IVD, IVD|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP2974a was selected from the N-term region of human IVD. 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.|
|Cellular Location||Mitochondrion matrix.|
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Provided below are standard protocols that you may find useful for product applications.
Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia.
Vockley,J., et.al., Am. J. Hum. Genet. 49 (1), 147-157 (1991)Kraus,J.P., et.al., Genomics 1 (3), 264-269 (1987)
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