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Chemical Compound Review

AR-1H5730     [5-(6-aminopurin-9-yl)-4- hydroxy-2...

Synonyms: AC1L1A2Q, AC1Q68ZC, Coenzyme A, valeryl-, Coenzyme A, S-pentanoate
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Disease relevance of isovaleryl-CoA

  • Furthermore, it was postulated that iso-FAs or compounds derived thereof are involved in fruiting body formation in Myxococcus xanthus, since mutants with a reduced level of iso-FA due to a reduced level of the precursor isovaleryl-CoA, are delayed in aggregation and produce only few myxospores [1].
  • A biosynthetic pathway to isovaleryl-CoA in myxobacteria: the involvement of the mevalonate pathway [2].

High impact information on isovaleryl-CoA


Biological context of isovaleryl-CoA


Anatomical context of isovaleryl-CoA


Associations of isovaleryl-CoA with other chemical compounds

  • The products of SCA dehydrogenase/butyryl-CoA, MCA dehydrogenase/octanoyl-CoA, and IV dehydrogenase/isovaleryl-CoA reactions were identified as crotonyl-CoA, 2-octenoyl-CoA, and 3-methylcrotonyl-CoA, respectively, using gas chromatography [14].
  • This resistance is not due to weak binding: the complex between isovaleryl-CoA dehydrogenase and 2-pentynoyl-CoA shows a Kd of 1.8 microM at pH 7 [15].
  • We developed a modified version of the tritium release assay to accurately measure residual isovaleryl-CoA dehydrogenase activity in fibroblasts from patients with both forms of isovaleric acidemia [16].
  • In order to begin to identify the role of AidB in the cell, the protein was purified to homogeneity, shown to possess stoichiometric amounts of flavin adenine dinucleotide (FAD), and confirmed to have low levels of isovaleryl-coenzyme A (CoA) dehydrogenase activity [17].
  • Isovaleryl-CoA dehydrogenase (IVD) is a homotetrameric flavoenzyme, which catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA and transfers electrons to the electron-transferring flavoprotein, and is a member of the acyl-CoA dehydrogenase (ACD) enzyme family [18].

Gene context of isovaleryl-CoA


Analytical, diagnostic and therapeutic context of isovaleryl-CoA


  1. Novel Iso-branched Ether Lipids as Specific Markers of Developmental Sporulation in the Myxobacterium Myxococcus xanthus. Ring, M.W., Schw??r, G., Thiel, V., Dickschat, J.S., Kroppenstedt, R.M., Schulz, S., Bode, H.B. J. Biol. Chem. (2006) [Pubmed]
  2. A biosynthetic pathway to isovaleryl-CoA in myxobacteria: the involvement of the mevalonate pathway. Mahmud, T., Wenzel, S.C., Wan, E., Wen, K.W., Bode, H.B., Gaitatzis, N., Müller, R. Chembiochem (2005) [Pubmed]
  3. Molecular heterogeneity of variant isovaleryl-CoA dehydrogenase from cultured isovaleric acidemia fibroblasts. Ikeda, Y., Keese, S.M., Tanaka, K. Proc. Natl. Acad. Sci. U.S.A. (1985) [Pubmed]
  4. Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia. Rhead, W.J., Tanaka, K. Proc. Natl. Acad. Sci. U.S.A. (1980) [Pubmed]
  5. Isovaleryl-CoA dehydrogenase: demonstration in rat liver mitochondria by ion exchange chromatography and isoelectric focusing. Noda, C., Rhead, W.J., Tanaka, K. Proc. Natl. Acad. Sci. U.S.A. (1980) [Pubmed]
  6. Convergent evolution of a 2-methylbutyryl-CoA dehydrogenase from isovaleryl-CoA dehydrogenase in Solanum tuberosum. Goetzman, E.S., Mohsen, A.W., Prasad, K., Vockley, J. J. Biol. Chem. (2005) [Pubmed]
  7. Structures of isobutyryl-CoA dehydrogenase and enzyme-product complex: comparison with isovaleryl- and short-chain acyl-CoA dehydrogenases. Battaile, K.P., Nguyen, T.V., Vockley, J., Kim, J.J. J. Biol. Chem. (2004) [Pubmed]
  8. The variant human isovaleryl-CoA dehydrogenase gene responsible for type II isovaleric acidemia determines an RNA splicing error, leading to the deletion of the entire second coding exon and the production of a truncated precursor protein that interacts poorly with mitochondrial import receptors. Vockley, J., Nagao, M., Parimoo, B., Tanaka, K. J. Biol. Chem. (1992) [Pubmed]
  9. Molecular cloning and nucleotide sequence of cDNAs encoding the precursors of rat long chain acyl-coenzyme A, short chain acyl-coenzyme A, and isovaleryl-coenzyme A dehydrogenases. Sequence homology of four enzymes of the acyl-CoA dehydrogenase family. Matsubara, Y., Indo, Y., Naito, E., Ozasa, H., Glassberg, R., Vockley, J., Ikeda, Y., Kraus, J., Tanaka, K. J. Biol. Chem. (1989) [Pubmed]
  10. Leucine catabolism during the differentiation of 3T3-L1 cells. Expression of a mitochondrial enzyme system. Frerman, F.E., Sabran, J.L., Taylor, J.L., Grossberg, S.E. J. Biol. Chem. (1983) [Pubmed]
  11. Isolation of cDNA clones coding for rat isovaleryl-CoA dehydrogenase and assignment of the gene to human chromosome 15. Kraus, J.P., Matsubara, Y., Barton, D., Yang-Feng, T.L., Glassberg, R., Ito, M., Ikeda, Y., Mole, J., Francke, U., Tanaka, K. Genomics (1987) [Pubmed]
  12. Stable isotope dilution analysis of isovalerylglycine in amniotic fluid and urine and its application for the prenatal diagnosis of isovaleric acidemia. Hine, D.G., Hack, A.M., Goodman, S.I., Tanaka, K. Pediatr. Res. (1986) [Pubmed]
  13. Establishment of a practical enzymatic assay method for determination of isovaleryl-CoA dehydrogenase activity using high-performance liquid chromatography. Tajima, G., Sakura, N., Yofune, H., Dwi Bahagia Febriani, A., Nishimura, Y., Sakamoto, A., Ono, H., Shigematsu, Y., Kobayashi, M. Clin. Chim. Acta (2005) [Pubmed]
  14. Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver. Finocchiaro, G., Ito, M., Tanaka, K. J. Biol. Chem. (1987) [Pubmed]
  15. Mechanism-based inhibitor discrimination in the acyl-CoA dehydrogenases. Schaller, R.A., Mohsen, A.W., Vockley, J., Thorpe, C. Biochemistry (1997) [Pubmed]
  16. Isovaleryl-CoA dehydrogenase activity in isovaleric acidemia fibroblasts using an improved tritium release assay. Hyman, D.B., Tanaka, K. Pediatr. Res. (1986) [Pubmed]
  17. The AidB component of the Escherichia coli adaptive response to alkylating agents is a flavin-containing, DNA-binding protein. Rohankhedkar, M.S., Mulrooney, S.B., Wedemeyer, W.J., Hausinger, R.P. J. Bacteriol. (2006) [Pubmed]
  18. Arginine 387 of human isovaleryl-CoA dehydrogenase plays a crucial role in substrate/product binding. Volchenboum, S.L., Mohsen, A.W., Kim, J.J., Vockley, J. Mol. Genet. Metab. (2001) [Pubmed]
  19. Identification of the active site catalytic residue in human isovaleryl-CoA dehydrogenase. Mohsen, A.W., Vockley, J. Biochemistry (1995) [Pubmed]
  20. Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands. Searls, T., Butler, D., Chien, W., Mukherji, M., Lloyd, M.D., Schofield, C.J. J. Lipid Res. (2005) [Pubmed]
  21. Identification of Caenorhabditis elegans isovaleryl-CoA dehydrogenase and structural comparison with other acyl-CoA dehydrogenases. Mohsen, A.W., Navarette, B., Vockley, J. Mol. Genet. Metab. (2001) [Pubmed]
  22. Metabolism of leucine in fibroblasts from patients with deficiencies in each of the major catabolic enzymes: branched-chain ketoacid dehydrogenase, isovaleryl-CoA dehydrogenase, 3-methylcrotonyl-CoA carboxylase, 3-methylglutaconyl-CoA hydratase, and 3-hydroxy-3-methylglutaryl-CoA lyase. Yoshida, I., Søvik, O., Sweetman, L., Nyhan, W.L. J. Neurogenet. (1985) [Pubmed]
  23. Selective inactivation of various acyl-CoA dehydrogenases by (methylenecyclopropyl)acetyl-CoA. Ikeda, Y., Tanaka, K. Biochim. Biophys. Acta (1990) [Pubmed]
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