The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

Ivd  -  isovaleryl-CoA dehydrogenase

Rattus norvegicus

Synonyms: IVD, Isovaleryl-CoA dehydrogenase, mitochondrial
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Ivd

  • The most prominent biochemical consequence of riboflavin deficiency in rats is a drastic decrease in various acyl-CoA dehydrogenase activities, especially that of short chain and isovaleryl-CoA dehydrogenase (IVD) [1].
  • The overlapping cDNAs encode a polypeptide of 604 amino acids (aa) with a predicted M(r) of 68,502, which is 91.4% identical to the rat homolog, rPDE IVD. hPDE IVD produced in Escherichia coli was inhibited by rolipram [2].

High impact information on Ivd

  • We have reported elsewhere nine point mutations in the IVD gene in fibroblasts of patients with IVA, which lead to abnormalities in IVD protein processing and activity [3].
  • Exon skipping in IVD RNA processing in isovaleric acidemia caused by point mutations in the coding region of the IVD gene [3].
  • Dexamethasone induced IVD mRNA in both the liver and heart [4].
  • In contrast, it strongly suppressed mRNAs of all ACDs and alpha-ETF mRNA in the liver, except IVD mRNA [4].
  • cDNAs encoding the entire coding regions of the precursors (p) of rat long chain acyl-CoA (LCAD), short chain acyl-CoA (SCAD) and isovaleryl-CoA dehydrogenase (IVD) have been cloned and sequenced [5].

Biological context of Ivd

  • cDNAs encoding human family-IV phosphodiesterase, subtype D (hPDE IVD) were isolated from a human heart cDNA library [2].
  • We reported previously that OA induces neuronal apoptosis of immature neurons (in vitro days (IVD) 3-5), which is inhibited by cycloheximide (CHX) [6].

Anatomical context of Ivd

  • Expression of the hPDE IVD mRNA is widespread in human tissues and most abundant in skeletal muscle [2].

Associations of Ivd with chemical compounds

  • The position of the catalytic glutamate, identified as Glu376 in porcine medium chain acyl-CoA dehydrogenase (MCAD), Glu254 in human isovaleryl-CoA dehydrogenase (IVD), and Glu261 in human long chain acyl-CoA dehydrogenase (LCAD), has been suggested to affect substrate chain length specificity [7].
  • Isolation of a cDNA encoding a human rolipram-sensitive cyclic AMP phosphodiesterase (PDE IVD) [2].
  • In this study, we demonstrate that CHX fails to prevent OA-induced neuronal death in mature neurons (IVD 14-15) [6].
  • Liposoluble extracts of serum from healthy men and AIDS patients (stages IVC1 and IVD by CDC criteria) inhibited the incorporation of [3H]thymidine into isolated rat thymocytes, but AIDS extracts were less inhibitory, requiring 1.8 times more cortisol in the AIDS extracts than in the healthy extracts to inhibit [3H]thymidine incorporation by 50% [8].

Analytical, diagnostic and therapeutic context of Ivd

  • Feeding the rats a fat-free diet for 7 days caused a marked increase of IVD mRNA in the heart, whereas the high fat diet for the same period resulted in a severe decrease of the same degree, suggesting a protein-sparing mechanism [4].


  1. FAD-dependent regulation of transcription, translation, post-translational processing, and post-processing stability of various mitochondrial acyl-CoA dehydrogenases and of electron transfer flavoprotein and the site of holoenzyme formation. Nagao, M., Tanaka, K. J. Biol. Chem. (1992) [Pubmed]
  2. Isolation of a cDNA encoding a human rolipram-sensitive cyclic AMP phosphodiesterase (PDE IVD). Baecker, P.A., Obernolte, R., Bach, C., Yee, C., Shelton, E.R. Gene (1994) [Pubmed]
  3. Exon skipping in IVD RNA processing in isovaleric acidemia caused by point mutations in the coding region of the IVD gene. Vockley, J., Rogan, P.K., Anderson, B.D., Willard, J., Seelan, R.S., Smith, D.I., Liu, W. Am. J. Hum. Genet. (2000) [Pubmed]
  4. Developmental, nutritional, and hormonal regulation of tissue-specific expression of the genes encoding various acyl-CoA dehydrogenases and alpha-subunit of electron transfer flavoprotein in rat. Nagao, M., Parimoo, B., Tanaka, K. J. Biol. Chem. (1993) [Pubmed]
  5. 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]
  6. Differential activation of caspase-3 at two maturational stages during okadaic acid-induced rat neuronal death. Hong, H.N., Yoon, S.Y., Suh, J., Lee, J.H., Kim, D. Neurosci. Lett. (2002) [Pubmed]
  7. Functional role of the active site glutamate-368 in rat short chain acyl-CoA dehydrogenase. Battaile, K.P., Mohsen, A.W., Vockley, J. Biochemistry (1996) [Pubmed]
  8. Perturbation of the immunosuppressive action of glucocorticoids in rat thymocytes by liposoluble extracts of serum from AIDS patients. Sumida, C., Vallette, G., Thobie, N., Nunez, E.A. AIDS Res. Hum. Retroviruses (1993) [Pubmed]
WikiGenes - Universities