Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

Official Name

5-aminolevulinate synthase

Name from literature

5-aminolevulinate synthase

Pathway from literature

hepatic heme biosynthesis

Pathway from KEGG

Amino Acid Metabolism; Glycine, serine and threonine metabolism; map00260

Metabolism of Cofactors and Vitamins; Porphyrin and chlorophyll metabolism; map00860


Mouse (10090)

Genome localization

X F3|X 63.0 cM[11656 ], 9 F1[11655 ],


A pyridoxal-phosphate protein. The enzyme in erythrocytes is genetically distinct from that in other tissues.

Rate-limiting Description

"The erythroid-specific isoform of 5-aminolevulinate synthase (ALAS2) catalyzes the rate-limiting step in heme biosynthesis." (12393745)

Regulatory Information

Regulatory type




transcriptional factor;

"Experimental validation of predicted mammalian erythroid cis-regulatory modules." (17038566)

Gene ontology

Gene ontology

GO:0001666 (P) response to hypoxia [P08680 ];
GO:0006783 (P) heme biosynthetic process [P08680, Q8VC19 ];
GO:0016769 (F) transferase activity, transferring nitrogen... [P08680, Q8VC19 ];
GO:0003870 (F) 5-aminolevulinate synthase activity [P08680, Q8VC19 ];
GO:0030170 (F) pyridoxal phosphate binding [P08680, Q8VC19 ];
GO:0005759 (C) mitochondrial matrix [P08680, Q8VC19 ];
GO:0006879 (P) cellular iron ion homeostasis [P08680 ];
GO:0005515 (F) protein binding [P08680 ];
GO:0005743 (C) mitochondrial inner membrane [P08680 ];
GO:0030218 (P) erythrocyte differentiation [P08680 ];
GO:0042541 (P) hemoglobin biosynthetic process [P08680 ];

Subcellular localization


mitochondrion [P08680, Q8VC19 ];



P08680; Q8VC19

Entrez Gene

11655; 11656

  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min