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Literature summary extracted from

  • Goetzman, E.S.; Wang, Y.; He, M.; Mohsen, A.W.; Ninness, B.K.; Vockley, J.
    Expression and characterization of mutations in human very long-chain acyl-CoA dehydrogenase using a prokaryotic system (2007), Mol. Genet. Metab., 91, 138-147.
    View publication on PubMedView publication on EuropePMC

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.3.8.8 expressed in Escherichia coli strain C43 (DE3) Homo sapiens
1.3.8.8 expressed in Escherichia coli strain C43(DE3) Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.3.8.8 A450P mutant shows normal wild type activity and greatly reduced mitochondrial membrane association Homo sapiens
1.3.8.8 A450P unpurified recombinant enzyme shows increased activity, pure mutant enzyme has wild type activity Homo sapiens
1.3.8.8 L462P in the pure form, mutant has roughly 30% of wild type activity and greatly reduced mitochondrial membrane association Homo sapiens
1.3.8.8 L462P unpurified recombinant enzyme shows increased activity, pure mutant enzyme has 30% activity compared to the wild type enzyme Homo sapiens
1.3.8.8 R429W mutant shows 0.2% of wild type activity, the mutation leads to a severe form of VLCAD deficiency Homo sapiens
1.3.8.8 R429W the mutation is observed in the severe form of VLCAD deficiency Homo sapiens
1.3.8.8 R573W no activity, the mutation leads to a severe form of VLCAD deficiency Homo sapiens
1.3.8.8 R573W shows 0.2% of wild type activity Homo sapiens
1.3.8.8 T220M mutant shows 3% of wild type activity, the mutation leads to a mild form of VLCAD deficiency Homo sapiens
1.3.8.8 T220M shows 3% of wild type activity, the mutation is observed in the mild form of VLCAD deficiency Homo sapiens
1.3.8.8 V243A mutant shows 22% of wild type activity, the mutation leads to a mild form of VLCAD deficiency Homo sapiens
1.3.8.8 V243A shows 22% of wild type activity, the mutation is observed in the mild form of VLCAD deficiency Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.3.8.8 mitochondrial membrane
-
 Homo sapiens 31966
-
1.3.8.8 mitochondrion inner mitochondrial membrane Homo sapiens 5739
-

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
1.3.8.8 120000
-
 HPLC gel filtration Homo sapiens

Organism

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

Purification (Commentary)

EC Number Purification (Comment) Organism
1.3.8.8 ammonium sulfate precipitation, DEAE Sepharose column chromatography, and SP Sepharose column chromatography Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.3.8.8 arachidonoyl-CoA + acceptor 48.9% activity compared to the wild type enzyme Homo sapiens ? + reduced acceptor
-
 ?
1.3.8.8 arachidonoyl-CoA + electron transfer flavoprotein 48.9% activity compared to palmitoyl-CoA Homo sapiens (2E,5Z,8Z,11Z,14Z)-2,5,8,11,14-eicosapentaenoyl-CoA + reduced electron transfer flavoprotein
-
 ?
1.3.8.8 lineoyl-CoA + acceptor 12.5% activity compared to the wild type enzyme Homo sapiens 2,9,12-octadecatrienoyl-CoA + reduced acceptor
-
 ?
1.3.8.8 linoleoyl-CoA + electron transfer flavoprotein 12.5% activity compared to palmitoyl-CoA Homo sapiens 2,9,12-octadecatrienoyl-CoA + reduced electron transfer flavoprotein
-
 ?
1.3.8.8 myristoyl-CoA + acceptor 51.1% activity compared to the wild type enzyme Homo sapiens ? + reduced acceptor
-
 ?
1.3.8.8 myristoyl-CoA + electron transfer flavoprotein 51.1% activity compared to palmitoyl-CoA Homo sapiens ? + reduced electron transfer flavoprotein
-
 ?
1.3.8.8 oleoyl-CoA + acceptor 40.8% activity compared to the wild type enzyme Homo sapiens 2,9-octadecadienoyl-CoA + reduced acceptor
-
 ?
1.3.8.8 oleoyl-CoA + electron transfer flavoprotein 40.8% activity compared to palmitoyl-CoA Homo sapiens 2,9-octadecadienoyl-CoA + reduced electron transfer flavoprotein
-
 ?
1.3.8.8 palmitoleoyl-CoA + acceptor 34.8% activity compared to the wild type enzyme Homo sapiens (2E,9Z)-2,9-hexadecadienoyl-CoA + reduced acceptor
-
 ?
1.3.8.8 palmitoleoyl-CoA + electron transfer flavoprotein 34.8% activity compared to palmitoyl-CoA Homo sapiens (2E,9Z)-2,9-hexadecadienoyl-CoA + reduced electron transfer flavoprotein
-
 ?
1.3.8.8 palmitoyl-CoA + electron transfer flavoprotein 100% activity with palmitoyl-CoA Homo sapiens 2-hexadecenoyl-CoA + reduced electron transfer flavoprotein
-
 ?
1.3.8.8 palmitoyl-CoA + electron-transfer flavoprotein 100% activity Homo sapiens (2E)-2-hexadecenoyl-CoA + reduced electron-transfer flavoprotein
-
 ?
1.3.8.8 stearoyl-CoA + acceptor 39% activity compared to the wild type enzyme Homo sapiens 2-octadecenoyl-CoA + reduced acceptor
-
 ?
1.3.8.8 stearoyl-CoA + electron transfer flavoprotein 39.0% activity compared to palmitoyl-CoA Homo sapiens 2-octadecenoyl-CoA + reduced electron transfer flavoprotein
-
 ?

Subunits

EC Number Subunits Comment Organism
1.3.8.8 homodimer
-
 Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
1.3.8.8 very long chain acyl-CoA dehydrogenase
-
 Homo sapiens
1.3.8.8 very long-chain acyl-CoA dehydrogenase
-
 Homo sapiens
1.3.8.8 VLCAD
-
 Homo sapiens
1.3.8.8 VLMCAD
-
 Homo sapiens