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Literature summary for 4.1.3.4 extracted from

  • Casals, N.; Gomez-Puertas, P.; Pie, J.; Mir, C.; Roca, R.; Puisac, B.; Aledo, R.; Clotet, J.; Menao, S.; Serra, D.; Asins, G.; Till, J.; Elias-Jones, A.C.; Cresto, J.C.; Chamoles, N.A.; Abdenur, J.E.; Mayatepek, E.; Besley, G.; Valencia, A.; Hegardt, F.G.
    Structural (betaalpha)8 TIM barrel model of 3-hydroxy-3-methylglutaryl-coenzyme A lyase (2003), J. Biol. Chem., 278, 29016-29023.
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Escherichia coli Homo sapiens

Protein Variants

Protein Variants Comment Organism
D204N reduced catalytic efficiency, mutation causes 3-hydroxy-3-methylgluratic aciduria in vivo Homo sapiens
S201Y no catalytic efficiency, mutation causes 3-hydroxy-3-methylgluratic aciduria in vivo Homo sapiens
S75R no catalytic efficiency, mutation causes 3-hydroxy-3-methylgluratic aciduria in vivo Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
3.1
-
(S)-3-hydroxy-3-methylglutaryl-CoA 37°C, pH 7.5, wild type Homo sapiens
6.6
-
(S)-3-hydroxy-3-methylglutaryl-CoA 37°C, pH 7.5, D204N mutant Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens last step in ketogenesis ?
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
Homo sapiens
-
lymphocyte
-
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
-
?
additional information last step in ketogenesis Homo sapiens ?
-
?