ALAS1 - 5-aminolevulinate synthase, nonspecific, mitochondrial - human protein (Function)
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ALAS1 »  5-aminolevulinate synthase, nonspecific, mitochondrial   [ EC ]  (ALAS-H)
Protein also known as:  Delta-aminolevulinate synthase 1.
Gene name:  ALAS1
Entry whose protein(s) existence is based on evidence at protein level
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GO molecular function 
5-aminolevulinate synthase activitydefinition[GO:0003870]  
  • IEAEC 2 GO
Pyridoxal phosphate bindingdefinition[GO:0030170] silver  
  • IEAInterPro 2 GO
GO biological process 
Protoporphyrinogen IX biosynthetic processdefinition[GO:0006782]  
  • IEAUniPathway
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC Succinyl-CoA + glycine 5-aminolevulinate + CoA + CO(2).  
  • CuratedUniProtKB
It requires the following cofactor
Pyridoxal phosphate.  
  • CuratedUniProtKB
This protein is involved in the following pathway
According to KEGG, this protein belongs to the following pathways:
Glycine, serine and threonine metabolism  hsa00260+211  
Metabolic pathways  hsa01100+211  
Porphyrin and chlorophyll metabolism  hsa00860+211  
According to Reactome, this protein belongs to the following pathways:
Heme biosynthesis  REACT_9465  
PPARA activates gene expression  REACT_116145  
Transcriptional activation of mitochondrial biogenesis  REACT_200608  
More information is available from:
There are two delta-ALA synthases in vertebrates: an erythroid- specific form and one (housekeeping) which is expressed in all tissues.  
  • CuratedUniProtKB


Biological process 
Heme biosynthesis  definition   [KW-0350]
Molecular function 
Acyltransferase  definition   [KW-0012]
Transferase  definition   [KW-0808]
Technical term 
Reference proteome  definition   [KW-1185]

Further external links

GeneWiki: ALAS1
GenomeRNAi: 211
PRO: PR:P13196