Literature summary for 4.1.3.4 extracted from

Pie, J.; Lopez-Vinas, E.; Puisac, B.; Menao, S.; Pie, A.; Casale, C.; Ramos, F.J.; Hegardt, F.G.; Gomez-Puertas, P.; Casals, N.
Molecular genetics of HMG-CoA lyase deficiency (2007), Mol. Genet. Metab., 92, 198-209.

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Protein Variants

Protein Variants	Comment	Organism
D204N	lack of enzymatic activity	Homo sapiens
D42H	lack of enzymatic activity	Homo sapiens
E279K	lack of enzymatic activity	Homo sapiens
G203E	lack of enzymatic activity	Homo sapiens
H233R	lack of enzymatic activity	Homo sapiens
K48N	lack of enzymatic activity	Homo sapiens
L263P	lack of enzymatic activity	Homo sapiens
R41Q	lack of enzymatic activity	Homo sapiens
S201Y	lack of enzymatic activity	Homo sapiens
S75R	lack of enzymatic activity	Homo sapiens
V70L	lack of enzymatic activity	Homo sapiens

Localization

Localization	Comment	Organism	GeneOntology No.	Textmining
mitochondrion	-	Homo sapiens	5739	-
peroxisome	-	Homo sapiens	5777	-

Metals/Ions

Metals/Ions	Comment	Organism	Structure
Mg2+	required for activity	Homo sapiens
Mn2+	required for activity	Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da]	Molecular Weight Maximum [Da]	Comment	Organism
31500	-	SDS-PAGE, mitochondrial enzyme	Homo sapiens
34100	-	SDS-PAGE, peroxisomal enzyme	Homo sapiens

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	P35914	-	-

Oxidation Stability

Oxidation Stability	Organism
the enzyme is sensitive to oxidation, showing higher activity in reducing conditions	Homo sapiens

Source Tissue

Source Tissue	Comment	Organism	Textmining
fibroblast	-	Homo sapiens	-
kidney	-	Homo sapiens	-
liver	-	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

pH Optimum

pH Optimum Minimum	pH Optimum Maximum	Comment	Organism
9	-	-	Homo sapiens

pI Value

Organism	Comment	pI Value Maximum	pI Value
Homo sapiens	mitochondrial enzyme, isoelectric focusing	-	6.2
Homo sapiens	peroxisomal enzyme, isoelectric focusing	-	7.6

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Release 2023.1 (January 2023)