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Literature summary for 1.3.8.4 extracted from

  • Mohsen, A.W.A.; Anderson, B.D.; Volchenboum, S.L.; Battaile, K.P.; Tiffany, K.; Roberts, D.; Kim, J.J.P.; Vockley, J.
    Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia (1998), Biochemistry, 37, 10325-10335.
    View publication on PubMed
Search for more literature for 1.3.8.4

Cloned(Commentary)

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

Protein Variants

Protein Variants Comment Organism
A282V enzyme activity detected, 19% relative activity to wild-type Homo sapiens
B40N has no detectable enzymatic activity Homo sapiens
C328R has no detectable enzymatic activity Homo sapiens
L13P has no detectable enzymatic activity Homo sapiens
R21P has no detectable enzymatic activity Homo sapiens
R363C enzyme activity detected, 1% relative activity to wild-type Homo sapiens
R382L enzyme activity detected, 7% relative activity to wild-type Homo sapiens
V342A enzyme activity detected, 12% relative activity to wild-type Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.0028
-
 isovaleryl-CoA purified enzyme V342A Homo sapiens
0.0028
-
 isovaleryl-CoA purified V342A mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
0.0031
-
 isovaleryl-CoA purified wild-type enzyme, electron acceptor: electron-transferring flavoprotein Homo sapiens
0.0045
-
 isovaleryl-CoA purified R382L mutant, electron acceptor: dichlorophenol indophenol Homo sapiens
0.0069
-
 isovaleryl-CoA purified R382L mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
0.024
-
 isovaleryl-CoA purified V342A mutant, electron acceptor: dichlorophenol indophenol Homo sapiens
0.027
-
 isovaleryl-CoA purified A282V mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
0.0296
-
 isovaleryl-CoA purified wild-type enzyme, electron acceptor: dichlorophenol indophenol Homo sapiens
0.41
-
 isovaleryl-CoA purified A282V mutant, electron acceptor: dichlorophenol indophenol Homo sapiens

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 deficiency of the enzyme in humans is responsible for isovaleric acidemia ?
-
 ?
additional information Homo sapiens the enzyme catalyzes the third step of the leucine oxidative metabolism ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens P26440
-
-

Purification (Commentary)

Purification (Comment) Organism
by loading the supernatant from an induced culture onto an XK16/20 Fast Q fast flow column, overnight dialysis in potassium phosphate buffer, and hydroxyapatite column chromatography 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
0.00034
-
 fibroblast cell line harboring the mutation corresponding to the A282V mutant Homo sapiens
0.004
-
 cell free extract of Escherichia coli cells expressing the enzyme mutant R363C Homo sapiens
0.0041
-
 normal cell lines Homo sapiens
0.032
-
 cell free extract of Escherichia coli cells expressing the enzyme mutant R382L Homo sapiens
0.052
-
 cell free extract of Escherichia coli cells expressing the enzyme mutant V342A Homo sapiens
0.086
-
 cell free extract of Escherichia coli cells expressing the enzyme mutant A282V Homo sapiens
0.44
-
 cell free extract of Escherichia coli cells expressing the enzyme, wild type Homo sapiens
0.6
-
 of purified recombinant V342A mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
1.24
-
 of purified recombinant V342A mutant, electron acceptor: dichlorophenol indophenol + FAD Homo sapiens
1.27
-
 of purified recombinant V342A mutant, electron acceptor: dichlorophenol indophenol Homo sapiens
1.4
-
 of purified recombinant R382L mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
2.04
-
 of purified recombinant A282V mutant, electron acceptor: dichlorophenol indophenol Homo sapiens
2.2
-
 of purified recombinant A282V mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
2.33
-
 of purified recombinant A282V mutant, electron acceptor: dichlorophenol indophenol + FAD Homo sapiens
4.88
-
 of purified recombinant R382L mutant, electron acceptor: dichlorophenol indophenol Homo sapiens
6.34
-
 of purified recombinant R382L mutant, electron acceptor: dichlorophenol indophenol + FAD Homo sapiens
8.2
-
 of purified recombinant wild-type enzyme, electron acceptor: dichlorophenol indophenol Homo sapiens
10.3
-
 of purified recombinant wild-type enzyme, electron acceptor: dichlorophenol indophenol + FAD Homo sapiens
11.7
-
 of purified recombinant wild-type enzyme, electron acceptor: electron-transferring flavoprotein 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 electron acceptor: electron-transferring flavoprotein, dichlorophenol indophenol or dichlorophenol indophenol + FAD Homo sapiens 3-methylcrotonyl-CoA + reduced acceptor
-
 ?
additional information deficiency of the enzyme in humans is responsible for isovaleric acidemia Homo sapiens ?
-
 ?
additional information the enzyme catalyzes the third step of the leucine oxidative metabolism Homo sapiens ?
-
 ?

Turnover Number [1/s]

Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
0.283
-
 isovaleryl-CoA purified V342A mutant, electron acceptor: dichlorophenol indophenol Homo sapiens
0.733
-
 isovaleryl-CoA purified R382L mutant, electron acceptor: dichlorophenol indophenol Homo sapiens
0.817
-
 isovaleryl-CoA purified V342A mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
2
-
 isovaleryl-CoA purified R382L mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
2.2
-
 isovaleryl-CoA purified A282V mutant, electron acceptor: electron-transferring flavoprotein Homo sapiens
2.82
-
 isovaleryl-CoA purified A282V mutant, electron acceptor: dichlorophenol indophenol Homo sapiens
8.8
-
 isovaleryl-CoA purified wild-type enzyme, electron acceptor: electron-transferring flavoprotein Homo sapiens
10
-
 isovaleryl-CoA purified wild-type enzyme, electron acceptor: dichlorophenol indophenol Homo sapiens