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Literature summary for 4.1.3.4 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)

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

Protein Variants

Protein Variants Comment Organism
K48N the mutation causes 3-hydroxy-3-methylglutaric aciduria, shows less than 2% of wild type HMG-CoA lyase activity Homo sapiens
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]

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

Localization

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

Organism

Organism UniProt Comment Textmining
Homo sapiens P35914
-
-

Purification (Commentary)

Purification (Comment) Organism
glutathione Sepharose column chromatography Homo sapiens

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
0.03
-
recombinant deletion mutant Mut15, at 37°C Homo sapiens
0.22
-
recombinant deletion mutant Mut12, at 37°C Homo sapiens
1.13
-
recombinant deletion mutant Mut3, at 37°C Homo sapiens
1.2
-
recombinant deletion mutant Mut6, at 37°C Homo sapiens
1.27
-
recombinant deletion mutant Mut9, at 37°C Homo sapiens
1.29
-
recombinant 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