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


Rat (10116)

Genome localization

-[65155 ], Xq21[25748 ],


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

Rate-limiting Description

"We show that the rate-limiting enzyme in hepatic heme biosynthesis, 5-aminolevulinate synthase (ALAS-1), is regulated by the peroxisome proliferator-activated receptor gamma coactivator 1alpha (PGC-1alpha)." (16122419)

"The first and rate-limiting step of heme biosynthesis is catalyzed by ALA synthase (ALA-S), the activity of which was determined in rat aorta using a radiometric assay, approximately 250 nmol x (g wet mass)(-1) x h(-1)." (15181459)

"In this study, we examined expression of HO-1 as well as non-specific delta-aminolevulinate synthase (ALAS1), the rate-limiting enzyme in heme catabolism and biosynthesis, respectively, in a rat model of PH produced by subcutaneous injection of MCT (60 mg/kg)." (16181105)

Gene ontology

Gene ontology

GO:0016594 (F) glycine binding [Q9ESE3 ];
GO:0001666 (P) response to hypoxia [Q63147, Q9ESE3 ];
GO:0005739 (C) mitochondrion [Q9ESE3 ];
GO:0006783 (P) heme biosynthetic process [Q63147, P13195 ];
GO:0016769 (F) transferase activity, transferring nitrogen... [Q63147, P13195 ];
GO:0050662 (F) coenzyme binding [Q9ESE3 ];
GO:0003870 (F) 5-aminolevulinate synthase activity [Q63147, P13195, Q9ESE3 ];
GO:0030170 (F) pyridoxal phosphate binding [Q63147, P13195 ];
GO:0005759 (C) mitochondrial matrix [Q63147, P13195 ];
GO:0030218 (P) erythrocyte differentiation [Q63147 ];
GO:0005515 (F) protein binding [Q63147 ];
GO:0005743 (C) mitochondrial inner membrane [Q63147 ];
GO:0042541 (P) hemoglobin biosynthetic process [Q63147 ];

Subcellular localization


mitochondrion [Q63147, P13195 ];



P13195; Q63147; Q9ESE3

Entrez Gene

25748; 65155

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