Literature summary for 2.3.1.37 extracted from

Fratz-Berilla, E.J.; Breydo, L.; Gouya, L.; Puy, H.; Uversky, V.N.; Ferreira, G.C.
Isoniazid inhibits human erythroid 5-aminolevulinate synthase Molecular mechanism and tolerance study with four X-linked protoporphyria patients (2017), Biochim. Biophys. Acta, 1863, 428-439 .

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Cloned(Commentary)

Cloned (Comment)	Organism
expressed in Escherichia coli BL21(DE3) cells	Homo sapiens

Inhibitors

Inhibitors	Comment	Organism	Structure
1,1-Dimethylhydrazine	about 75% residual activity at 0.01 mM	Homo sapiens
isoniazid	about 25% residual activity at 0.01 mM. Isoniazid directly inhibits the enzyme, noncompetitively or uncompetitively. about 0.4 mM pyridoxal 5'-phosphate and PMP reduce the effect of isoniazid on isoform ALAS2 inhibition	Homo sapiens
Pyrazinamide	about 85% residual activity at 0.01 mM	Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
succinyl-CoA + glycine	Homo sapiens	-	5-aminolevulinate + CoA + CO2	-	?

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	P22557	-	-

Purification (Commentary)

Purification (Comment)	Organism
nickel affinity chromatography column chromatography	Homo sapiens

Source Tissue

Source Tissue	Comment	Organism	Textmining
erythroid cell	-	Homo sapiens	-
HeLa cell	-	Homo sapiens	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
succinyl-CoA + glycine	-	Homo sapiens	5-aminolevulinate + CoA + CO2	-	?

Subunits

Subunits	Comment	Organism
?	x * 57300, calculated from amino acid sequence	Homo sapiens

Synonyms

Synonyms	Comment	Organism
ALAS2	-	Homo sapiens

Cofactor

Cofactor	Comment	Organism	Structure
pyridoxal 5'-phosphate	-	Homo sapiens

Ki Value [mM]

Ki Value [mM]	Ki Value maximum [mM]	Inhibitor	Comment	Organism	Structure
0.0012	-	isoniazid	apparent value, pH and temperature not specified in the publication	Homo sapiens

General Information

General Information	Comment	Organism
malfunction	enzyme mutations are associated with two different blood disorders, X-linked sideroblastic anemia and X-linked protoporphyria	Homo sapiens

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Release 2023.1 (January 2023)