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Literature summary extracted from

  • Zschocke, J.; Ruiter, J.P.N.; Brand, J.; Lindner, M.; Hoffmann, G.F.; Wanders, R.J.A.; Mayatepek, E.
    Progressive infantile neurodegeneration caused by 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency: a novel inborn error of branched-chain fatty acid and isoleucine metabolism (2000), Pediatr. Res., 48, 852-855.
    View publication on PubMed

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.1.1.178 2-methyl-3-hydroxybutyryl-CoA + NAD+ Homo sapiens isoleucine metabolism 2-methylacetoacetyl-CoA + NADH
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 ?
1.1.1.178 additional information Homo sapiens genetic deficiency of enzyme activity leads to neurodegenerative disease, that can be lessened by dietary isoleucine restriction, detection of high urinary levels of 2-methyl-3-hydroxybutyryl-CoA and tiglyl-glycine ?
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 ?

Organism

EC Number Organism UniProt Comment Textmining
1.1.1.178 Homo sapiens
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-
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Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.1.1.178 fibroblast
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 Homo sapiens
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Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
1.1.1.178 additional information
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 remaining enzyme activity in genetic deficient fibroblast is 0.02 nmol per min and mg protein Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.1.1.178 2-methyl-3-hydroxybutyryl-CoA + NAD+ isoleucine metabolism Homo sapiens 2-methylacetoacetyl-CoA + NADH
-
 ?
1.1.1.178 additional information genetic deficiency of enzyme activity leads to neurodegenerative disease, that can be lessened by dietary isoleucine restriction, detection of high urinary levels of 2-methyl-3-hydroxybutyryl-CoA and tiglyl-glycine Homo sapiens ?
-
 ?