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Literature summary for 1.3.8.4 extracted from

  • Mohsen, A.W.A.; Vockley, J.
    Identification of the active site catalytic residue in human isovaleryl-CoA dehydrogenase (1995), Biochemistry, 34, 10146-10152.
    View publication on PubMed
Search for more literature for 1.3.8.4

Cloned(Commentary)

Cloned (Comment) Organism
cDNA modified at its 5'-end coding region to enhance expression in Escherichia coli Homo sapiens

Protein Variants

Protein Variants Comment Organism
E254D has residual activity for isovaleryl-CoA, below 0.1% Homo sapiens
E254G has no detectable enzymatic activity Homo sapiens
E254G/A375E exhibits catalytic activity toward isovaleryl-CoA Homo sapiens
E254Q has no detectable enzymatic activity Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
isovaleryl-CoA + acceptor Homo sapiens
-
 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
additional information Homo sapiens deficiency of the enzyme in humans is responsible for isovaleric acidemia ?
-
 ?
additional information Homo sapiens E254 of the enzyme is in close proximity to the bound FAD, E254 is the active site catalytic residue ?
-
 ?
additional information Homo sapiens the enzyme catalyzes the third step of the leucine oxidative metabolism ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
0.08
-
 substrate: octanoyl-CoA Homo sapiens
1.19
-
 substrate: hexanoyl-CoA Homo sapiens
1.71
-
 substrate: butyryl-CoA Homo sapiens
3.71
-
 substrate: valeryl-CoA Homo sapiens
8.1
-
 substrate: isovaleryl-CoA Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
butyryl-CoA + acceptor 21% relative specific activity to isovaleryl-CoA Homo sapiens but-2-enoyl-CoA + reduced acceptor
-
 ?
hexanoyl-CoA + acceptor 15% relative specific activity to isovaleryl-CoA Homo sapiens hex-2-enoyl-CoA + reduced acceptor
-
 ?
isovaleryl-CoA + acceptor
-
 Homo sapiens 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
isovaleryl-CoA + acceptor the catalytic activity of the recombinant wild-type enzyme is the highest in the presence of isovaleryl-CoA Homo sapiens 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
isovaleryl-CoA + acceptor acceptors: electron-transfer flavoprotein, phenazine methosulfate Homo sapiens 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
additional information deficiency of the enzyme in humans is responsible for isovaleric acidemia Homo sapiens ?
-
 ?
additional information E254 of the enzyme is in close proximity to the bound FAD, E254 is the active site catalytic residue Homo sapiens ?
-
 ?
additional information the enzyme catalyzes the third step of the leucine oxidative metabolism Homo sapiens ?
-
 ?
n-valeryl-CoA + acceptor 46% relative specific activity to isovaleryl-CoA Homo sapiens pent-2-enoyl-CoA + reduced acceptor
-
 ?
octanoyl-CoA + acceptor 1% relative specific activity to isovaleryl-CoA Homo sapiens oct-2-enoyl-CoA + reduced acceptor
-
 ?

Cofactor

Cofactor Comment Organism Structure
FAD
-
 Homo sapiens