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Literature summary 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)

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

Protein Variants

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

Organism

EC Number Organism UniProt Comment Textmining
4.1.3.4 Homo sapiens P35914 mitochondrial precursor
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
4.1.3.4 9.5
-
wild type enzyme, at 37°C Homo sapiens

Substrates and Products (Substrate)

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

Synonyms

EC Number Synonyms Comment Organism
4.1.3.4 3-hydroxy-3-methylglutarate-CoA lyase
-
Homo sapiens
4.1.3.4 HMG-CoA lyase
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Homo sapiens
4.1.3.4 HMGL
-
Homo sapiens