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2-methyl-3-hydroxybutyryl-coenzyme A dehydrogenase (2-methyl-3-hydroxybutyryl-CoA dehydrogenase)

reaction:(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD+ = 2-methylacetoacetyl-CoA + NADH; deficiency is associated with progressive infantile neurodegeneration
Also Known As:
2-methyl-3-hydroxybutyryl-CoA dehydrogenase; (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD+ - oxidoreductase; 2-MHB-CoA dehydrogenase
Networked: 3 relevant articles (0 outcomes, 0 trials/studies)

Bio-Agent Context: Research Results

Experts

1. Ofman, Rob: 2 articles (09/2005 - 04/2004)
2. Wanders, Ronald J A: 2 articles (09/2005 - 04/2004)
3. Korman, Stanley H: 1 article (12/2006)
4. Perez-Cerda, Celia: 1 article (09/2005)
5. Merinero, Begoña: 1 article (09/2005)
6. Ramos, Julio: 1 article (09/2005)
7. Ribes, Antonia: 1 article (09/2005)
8. García-Villoria, Judit: 1 article (09/2005)
9. Sala, Pedro Ruiz: 1 article (09/2005)
10. Ugarte, Magdalena: 1 article (09/2005)

Related Diseases

1. Mitochondrial Diseases (Mitochondrial Disease)
2. Neurologic Manifestations (Neurological Manifestations)
3. Cerebral Palsy (Spastic Diplegia)

Related Drugs and Biologics

1. Isoleucine (L-Isoleucine)
2. tebufenozide (Mimic)
3. 2-methylacyl-CoA dehydrogenase