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Literature summary for 1.3.8.4 extracted from

  • Volchenboum, S.L.; Vockley, J.
    Mitochondrial import and processing of wild type and type III mutant isovaleryl-CoA dehydrogenase (2000), J. Biol. Chem., 275, 7958-7963.
    View publication on PubMed
Search for more literature for 1.3.8.4

Cloned(Commentary)

Cloned (Comment) Organism
expression of wild-type and mutants in Escherichia coli Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Homo sapiens 5739
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
170000
-
 gel filtration 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 the type III enzyme mutation responsible for isovaleric acidemia leads to a shift in reading frame and the subsequent incorporation of eight abnormally placed amino acids with premature termination of translation ?
-
 ?
additional information Homo sapiens a specific deficiency of enzyme activity is observed in cultured skin fibroblasts from patients with isovaleric acidemia ?
-
 ?
additional information Homo sapiens the enzyme catalyzes the third step of the leucine oxidative metabolism ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
0.0041
-
 normal cell lines Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
isovaleryl-CoA + acceptor
-
 Homo sapiens 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
additional information the type III enzyme mutation responsible for isovaleric acidemia leads to a shift in reading frame and the subsequent incorporation of eight abnormally placed amino acids with premature termination of translation Homo sapiens ?
-
 ?
additional information a specific deficiency of enzyme activity is observed in cultured skin fibroblasts from patients with isovaleric acidemia Homo sapiens ?
-
 ?
additional information the enzyme catalyzes the third step of the leucine oxidative metabolism Homo sapiens ?
-
 ?