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Literature summary extracted from

  • Carrasco, P.; Menao, S.; Lopez-Vinas, E.; Santpere, G.; Clotet, J.; Sierra, A.Y.; Gratacos, E.; Puisac, B.; Gomez-Puertas, P.; Hegardt, F.G.; Pie, J.; Casals, N.
    C-terminal end and amino acid Lys48 in HMG-CoA lyase are involved in substrate binding and enzyme activity (2007), Mol. Genet. Metab., 91, 120-127.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
4.1.3.4 expressed in Escherichia coli BL21 cells Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
4.1.3.4 K48N the mutation causes 3-hydroxy-3-methylglutaric aciduria, shows less than 2% of wild type HMG-CoA lyase activity Homo sapiens
4.1.3.4 additional information the deletion mutant Mut15 (last 15 C-terminal residues deleted) shows less than 2% specific activity, the deletion mutant Mut12 (last 12 C-terminal residues deleted) shows 17% specific activity, the deletion mutant Mut9 (last 9 C-terminal residues deleted) shows 99% specific activity, the deletion mutant Mut6 (last 6 C-terminal residues deleted) shows 93% specific activity, the deletion mutant Mut3 (last 3 C-terminal residues deleted) shows 88% specific activity, compared to the wild type enzyme, respectively Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
4.1.3.4 0.058
-
 (S)-3-hydroxy-3-methylglutaryl-CoA deletion mutant Mut15, at 37°C Homo sapiens
4.1.3.4 0.07
-
 (S)-3-hydroxy-3-methylglutaryl-CoA wild type enzyme, at 37°C Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
4.1.3.4 mitochondrion
-
 Homo sapiens 5739
-

Organism

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

Purification (Commentary)

EC Number Purification (Comment) Organism
4.1.3.4 glutathione Sepharose column chromatography Homo sapiens

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 0.03
-
 recombinant deletion mutant Mut15, at 37°C Homo sapiens
4.1.3.4 0.22
-
 recombinant deletion mutant Mut12, at 37°C Homo sapiens
4.1.3.4 1.13
-
 recombinant deletion mutant Mut3, at 37°C Homo sapiens
4.1.3.4 1.2
-
 recombinant deletion mutant Mut6, at 37°C Homo sapiens
4.1.3.4 1.27
-
 recombinant deletion mutant Mut9, at 37°C Homo sapiens
4.1.3.4 1.29
-
 recombinant 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
-
 Homo sapiens