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Literature summary for 1.1.1.31 extracted from

  • Loupatty, F.J.; van der Steen, A.; Ijlst, L.; Ruiter, J.P.; Ofman, R.; Baumgartner, M.R.; Ballhausen, D.; Yamaguchi, S.; Duran, M.; Wanders, R.J.
    Clinical, biochemical, and molecular findings in three patients with 3-hydroxyisobutyric aciduria (2006), Mol. Genet. Metab., 87, 243-248.
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.12
-
3-hydroxyisobutyric acid
-
Homo sapiens
0.5
-
NAD+
-
Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
affinity 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
0.0037
-
index patient with 3-hydroxyisobutyric acidia Homo sapiens
0.0042
-
patient 2 with 3-hydroxyisobutyric acidia Homo sapiens
0.0046
-
patient 1 with 3-hydroxyisobutyric acidia Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3-hydroxyisobutyric acid + NAD+
-
Homo sapiens methylmalonic semialdehyde + NADH
-
r

Synonyms

Synonyms Comment Organism
HIBADH
-
Homo sapiens

Cofactor

Cofactor Comment Organism Structure
NAD+
-
Homo sapiens