Literature summary 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.

Cloned(Commentary)

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

Protein Variants

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

KM Value [mM]

EC Number	KM Value [mM]	KM Value Maximum [mM]	Substrate	Comment	Organism	Structure
4.1.3.4	3.1	-	(S)-3-hydroxy-3-methylglutaryl-CoA	37°C, pH 7.5, wild type	Homo sapiens
4.1.3.4	6.6	-	(S)-3-hydroxy-3-methylglutaryl-CoA	37°C, pH 7.5, D204N mutant	Homo sapiens

Natural Substrates/ Products (Substrates)

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

Organism

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

Source Tissue

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

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Release 2023.1 (January 2023)