3-methylglutaconyl-CoA hydratase

3-meth·yl·glu·ta·con·yl-CoA hy·dra·tase

(meth'il-glū'tă-kon'il hī'dră-tās),
An enzyme that catalyzes the reaction of trans-3-methylglutaconyl-CoA and water to form 3-hydroxy-3-methylglutaconyl-CoA; this enzyme participates in the pathway for l-leucine degradation; a deficiency of this enzyme will result in 3-methylglutaconic aciduria.
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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.
3-Methylglutaconyl-CoA Hydratase Deficiency(*)--Fewer than 50 cases of this disorder have been identified.

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