IVD Antibody (N-term) Blocking Peptide
Synthetic peptide
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Primary Accession | P26440 |
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Clone Names | 81205066 |
Gene ID | 3712 |
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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 | Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed. |
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. |
Name | IVD (HGNC:6186) |
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Function | Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl- CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA (PubMed:7640268). |
Cellular Location | Mitochondrion matrix {ECO:0000250|UniProtKB:P12007} |
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Provided below are standard protocols that you may find useful for product applications.
Background
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.
References
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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