methylglutaconyl-CoA hydratase

catalyzes conversion of 3-hydroxy-3-methylglutaryl-CoA to trans-3-methylglutaconyl-CoA & water

Also Known As:

3-hydroxy-3-methylglutaryl-CoA hydro-lyase; 3-methylglutaconyl-CoA hydratase; 3-methylglutaconyl-coenzyme A hydratase

Networked: 3 relevant articles (0 outcomes, 0 trials/studies)

1.	Deodato, Federica: 1 article (08/2006)
2.	Wanders, Ronald J A: 1 article (08/2006)
3.	Tozzi, Giulia: 1 article (08/2006)
4.	Rizzo, Cristiano: 1 article (08/2006)
5.	Bertini, Enrico: 1 article (08/2006)
6.	Di Rosa, Gabriella: 1 article (08/2006)
7.	Carrozzo, Rosalba: 1 article (08/2006)
8.	Maiorana, Andrea: 1 article (08/2006)
9.	Loupatty, Ference J: 1 article (08/2006)
10.	Santorelli, Filippo M: 1 article (08/2006)

1.	Barth Syndrome 05/01/2006 - "3-Methylglutaconic aciduria type I, Barth syndrome, and Costeff syndrome were excluded as the activity of 3-methylglutaconyl-CoA hydratase, the cardiolipin levels, and molecular analysis of the OPA3 gene, respectively, showed no abnormalities. "
2.	Muscle Hypotonia (Hypotonia) 08/01/2006 - "We report five patients, all presenting a severe early-onset phenotype characterized by 3-methylglutaconic aciduria, hypertrophic cardiomyopathy, cataract, hypotonia/developmental delay, lactic acidosis, and normal 3-methylglutaconyl-CoA hydratase activity. "
3.	Metabolic Diseases (Metabolic Disease) 02/01/1999 - "3-Methylglutaconic (3-MGC) aciduria with 3-methylglutaconyl-CoA hydratase deficiency (3-MGC aciduria type I) is a rare inherited metabolic disease of L-leucine catabolism. "
4.	Cataract (Cataracts) 08/01/2006 - "We report five patients, all presenting a severe early-onset phenotype characterized by 3-methylglutaconic aciduria, hypertrophic cardiomyopathy, cataract, hypotonia/developmental delay, lactic acidosis, and normal 3-methylglutaconyl-CoA hydratase activity. "
5.	Hypertrophic Cardiomyopathy (Asymmetric Septal Hypertrophy) 08/01/2006 - "We report five patients, all presenting a severe early-onset phenotype characterized by 3-methylglutaconic aciduria, hypertrophic cardiomyopathy, cataract, hypotonia/developmental delay, lactic acidosis, and normal 3-methylglutaconyl-CoA hydratase activity. "

1.	Leucine (L-Leucine)
2.	Cardiolipins (Cardiolipin)
3.	Costeff optic atrophy syndrome