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The condensation product of isovaleric acid and coenzyme A; an intermediate in the catabolism of l-leucine.
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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.
[]/ [K.sub.m] [K.sub.m] ([micro][M.sup.-1] Enzyme Substrate ([micro]M) x [s.sup.-1]) ACS1-A acetyl-CoA 137 [+ or -] 91 0.140 ACS2-A acetyl-CoA 350 [+ or -] 2 0.114 ACS2-A isovaleryl-CoA 466 [+ or -] 119 0.109 ACS2-A phenylacetyl-CoA 3320 [+ or -] 1320 0.150 ACS3-A acetyl-CoA 474 [+ or -] 165 0.012 ACS4-A acetyl-CoA 242 [+ or -] 46 0.119 ACS5-A acetyl-CoA 620 [+ or -] 1.0 0.112 ACS5-A isobutyryl-CoA 2730 [+ or -] 1080 0.083 ACS5-A isovaleryl-CoA 820 [+ or -] 407 0.120 Table 2: Nucleotide kinetics of ACS1 isoforms.
BckDH oxidatively decarboxylates [alpha]-KIC to form isovaleryl-CoA and allows entry of the carbon skeleton of the increased cellular leucine into the TCA cycle.
Depois que a leucina e metabolizada a KIC, e metabolizado em isovaleryl-CoA pela enzima desidrogenase nas mitocondrias, ou em beta-hidroxi-beta-metilbutirato (HMB) no citosol, pela enzima dioxigenase (Nissen, 1992).
Isovaleric academia (WA) is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of isovaleryl-CoA dehydrogenase.
The production of [H.sub.2]S likely causes selective inhibition of several short-chain acyl-CoA dehydrogenases, including isobutyryl-CoA and isovaleryl-CoA dehydrogenases, resulting in the production of isobutyrylglycine and isovalerylglycine, respectively.
Defects in isovaleryl-CoA, 2 methylbutyryl-CoA, and hexanoyl-CoA dehydrogenases cause accumulation of their respective CoA esters, which conjugate with glycine to form
Increased accumulation of IV-carnitine presumably results from increased accumulation of isovaleryl-CoA, which is the major targeted marker for isovaleric acidemia, a disorder of leucine metabolism, and also of multiple acyl-CoA dehydrogenation defects resulting from impaired electron transport (7, 8).
Isovaleric acidemia (IVA) is an autosomal recessive genetic disorder of the enzyme isovaleryl-CoA dehydrogenase, which is involved in leucine metabolism (1).