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Literature summary for 1.3.8.8 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
Search for more literature for 1.3.8.8

Cloned(Commentary)

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

Protein Variants

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

Localization

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

Molecular Weight [Da]

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

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

Substrates and Products (Substrate)

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

Subunits

Subunits Comment Organism
homodimer
-
 Homo sapiens

Synonyms

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