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Literature summary for 1.3.8.4 extracted from

  • Rhead, W.J.; Tanaka, K.
    Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia (1980), Proc. Natl. Acad. Sci. USA, 77, 580-583.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 1.3.8.4

Localization

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

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 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
0.000039
-
 mitochondria from isovaleric acidemia cells, 13% relative activity to normal cells, specific activity determined by tritium release assay Homo sapiens
0.000163
-
 mitochondria from isovaleric acidemia cells, 12% relative activity to normal cells, specific activity determined by dye reduction assay Homo sapiens
0.00031
-
 mitochondria from normal cells, specific activity determined by tritium release assay Homo sapiens
0.00131
-
 mitochondria from normal cells, specific activity determined by dye reduction assay 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
-
 ?
isovaleryl-CoA + acceptor acceptors: electron-transfer flavoprotein, phenazine methosulfate Homo sapiens 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
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 ?
-
 ?