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Literature summary 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)

EC Number Cloned (Comment) Organism
1.1.1.31 expressed in Escherichia coli Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
1.1.1.31 0.12
-
 3-hydroxyisobutyric acid
-
 Homo sapiens
1.1.1.31 0.5
-
 NAD+
-
 Homo sapiens

Organism

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

Purification (Commentary)

EC Number Purification (Comment) Organism
1.1.1.31 affinity chromatography Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.1.1.31 fibroblast
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
1.1.1.31 0.0037
-
 index patient with 3-hydroxyisobutyric acidia Homo sapiens
1.1.1.31 0.0042
-
 patient 2 with 3-hydroxyisobutyric acidia Homo sapiens
1.1.1.31 0.0046
-
 patient 1 with 3-hydroxyisobutyric acidia Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.1.1.31 3-hydroxyisobutyric acid + NAD+
-
 Homo sapiens methylmalonic semialdehyde + NADH
-
 r

Synonyms

EC Number Synonyms Comment Organism
1.1.1.31 HIBADH
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
1.1.1.31 NAD+
-
 Homo sapiens