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Literature summary extracted from

  • Volchenboum, S.L.; Mohsen, A.W.A.; Kim, J.J.P.; Vockley, J.
    Arginine 387 of human isovaleryl-CoA dehydrogenase plays a crucial role in substrate/product binding (2001), Mol. Genet. Metab., 74, 226-237.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.3.8.4 expression of normal and mutant enzymes in Escherichia coli Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.3.8.4 L383R/R387A has no detectable activity in crude cellular extracts Homo sapiens
1.3.8.4 R387A enzyme activity detected, the mutant is less able than the mutant R387K to properly form the charge-transfer complex intermediate Homo sapiens
1.3.8.4 R387E enzyme activity detected, the mutant is less able than the mutant R387K to properly form the charge-transfer complex intermediate Homo sapiens
1.3.8.4 R387K enzyme activity detected, the mutant is able to form the charge-transfer complex intermediate with similar efficiency to wild-type Homo sapiens
1.3.8.4 R387Q enzyme activity detected, the mutant is less able than the mutant R387K to properly form the charge-transfer complex intermediate Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.3.8.4 0.0015
-
 isovaleryl-CoA wild-type Homo sapiens
1.3.8.4 0.0203
-
 isovaleryl-CoA mutant R387K Homo sapiens
1.3.8.4 0.0756
-
 isovaleryl-CoA mutant R387A Homo sapiens
1.3.8.4 0.195
-
 isovaleryl-CoA mutant R387Q Homo sapiens
1.3.8.4 0.9
-
 isovaleryl-CoA mutant R387E Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
1.3.8.4 isovaleryl-CoA + acceptor Homo sapiens
-
 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
1.3.8.4 additional information Homo sapiens R387 has an important role in anchoring the substrate ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
1.3.8.4 Homo sapiens
-
-
-

Purification (Commentary)

EC Number Purification (Comment) Organism
1.3.8.4 using bacterial lysates through centrifugation, filtration through an Acrodisc filter, chromatography on a XK16/20 DEAE fast-flow column and hydroxyapatite resin column Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.3.8.4 isovaleryl-CoA + acceptor
-
 Homo sapiens 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
1.3.8.4 isovaleryl-CoA + acceptor electron acceptor: electron-transferring flavoprotein Homo sapiens 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
1.3.8.4 additional information R387 has an important role in anchoring the substrate Homo sapiens ?
-
 ?