IVD

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IVD

(Interactive VideoDisc) See interactive video.
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Enzyme defects indicated by solid bars are numbered as follows: (1), branched-chain 2-ketoacid dehydrogenase complex deficiency (MSUD, maple syrup urine disease); (2), isovaleryl-CoA dehydrogenase deficiency (isovaleric acidemia); (3), isolated 3-methylcrotonyl-CoA carboxylase deficiency; (4), 3-methylcrotonyl-CoA carboxylase deficiency as part of a multiple carboxylase deficiency due to nutritional biotin deficiency or biotinidase deficiency or holocarboxylase synthetase deficiency or carbonic anhydrase VA deficiency or MT-ATP6 deficiency; (5), 3-methylglutaconyl-CoA hydratase deficiency; (6), 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) lyase deficiency.
IVA is a type of organic aciduria characterized by an abnormal leucine metabolism resulting from the deficiency in the enzyme isovaleryl-CoA dehydrogenase. The incidence is estimated to be 1 in 62.500-250.000 (5).
In MDM, IFN-[alpha] stimulation was associated with a downregulation of multiple genes associated with branched-chain amino acid catabolism including branched-chain aminotransferase 2 (BCAT2), isovaleryl-CoA dehydrogenase (IVD), hydroxyacyl-CoA dehydrogenase (HADH), and methylmalonyl-CoA epimerase (MCEE).
Lymphocyte isovaleryl-CoA dehydrogenase activity was 0.02 nmol/min.mg [controls mean [+ or -] SD: 1.51 [+ or -]0.31 nmol/min.mg].