ALA synthetase-1 (nonspecific 5-aminolevulinate synthetase mitochondrial, ALAS-H, ALAS1, ALAS3, ALASH, OK/SW-cl121)

Function

• cofactor biosynthesis; porphyrin biosynthesis
• 5-aminolevulinate from glycine: step 1/1

succinyl-CoA + glycine <--> 5-aminolevulinate + CoA + CO2

Cofactor: pyridoxal phosphate

Structure

• homodimer
• belongs to the class-2 pyridoxal-phosphate-dependent aminotransferase family

Compartment

mitochondrial matrix

Alternative splicing

named isoforms=2

Expression

expressed in all tissues

Notes

• there are 2 delta-ALA synthetase in vertebrates:
• an erythroid-specific form (ALAS2)
• a housekeeping form expressed in all tissues (ALAS1)

More general terms

• aminolevulinic acid [ALA] synthetase
• mitochondrial protein

Database

• UniProt: http://www.uniprot.org/uniprot/P13196.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:211
• OMIM: https://mirror.omim.org/entry/125290