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Literature summary 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.
    View publication on PubMed

Protein Variants

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

Localization

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

Metals/Ions

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

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
4.1.3.4 31500
-
 SDS-PAGE, mitochondrial enzyme Homo sapiens
4.1.3.4 34100
-
 SDS-PAGE, peroxisomal enzyme Homo sapiens

Organism

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

Oxidation Stability

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

Source Tissue

EC Number Source Tissue Comment Organism Textmining
4.1.3.4 fibroblast
-
 Homo sapiens
-
4.1.3.4 kidney
-
 Homo sapiens
-
4.1.3.4 liver
-
 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

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
4.1.3.4 9
-
-
 Homo sapiens

pI Value

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