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Literature summary for 4.1.3.4 extracted from

  • Mir, C.; Lopez-Vinas, E.; Aledo, R.; Puisac, B.; Rizzo, C.; Dionisi-Vici, C.; Deodato, F.; Pie, J.; Gomez-Puertas, P.; Hegardt, F.G.; Casals, N.
    A single-residue mutation, G203E, causes 3-hydroxy-3-methylglutaric aciduria by occluding the substrate channel in the 3D structural model of HMG-CoA lyase (2006), J. Inherit. Metab. Dis., 29, 64-70.
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli JM105 cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
G203E the mutation causes 3-hydroxy-3-methylglutaric aciduria, it has less than 0.02% of the wild type enzyme activity Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens P35914 mitochondrial precursor
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
9.5
-
wild type enzyme, at 37°C Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
(S)-3-Hydroxy-3-methylglutaryl-CoA
-
Homo sapiens Acetyl-CoA + acetoacetate
-
?

Synonyms

Synonyms Comment Organism
3-hydroxy-3-methylglutarate-CoA lyase
-
Homo sapiens
HMG-CoA lyase
-
Homo sapiens
HMGL
-
Homo sapiens