Function | Catalyzes the pyridoxal 5'-phosphate (PLP)-dependent condensation of succinyl-CoA and glycine to form aminolevulinic acid (ALA), with CoA and CO2 as by-products. Contributes significantly to heme formation during erythropoiesis. Isoform 3: Catalyzes the pyridoxal 5'-phosphate (PLP)-dependent condensation of succinyl-CoA and glycine to form aminolevulinic acid (ALA), with CoA and CO2 as by-products. Catalytic activity is 75-85% of isoform 1 activity. Isoform 4: Catalyzes the pyridoxal 5'-phosphate (PLP)-dependent condensation of succinyl-CoA and glycine to form aminolevulinic acid (ALA), with CoA and CO2 as by-products. Catalytic activity is 65-75% of isoform 1 activity. |
Protein Name | 5-Aminolevulinate Synthase - Erythroid-Specific - MitochondrialAlas-E5-Aminolevulinic Acid Synthase 2Delta-Ala Synthase 2Delta-Aminolevulinate Synthase 2 |
Database Links | Reactome: R-HSA-189451 |
Cellular Localisation | Mitochondrion Inner MembranePeripheral Membrane ProteinLocalizes To The Matrix Side Of The Mitochondrion Inner MembraneIsoform 4: Mitochondrion Inner Membrane |
Alternative Protein Names | 5-Aminolevulinate Synthase - Erythroid-Specific - Mitochondrial proteinAlas-E protein5-Aminolevulinic Acid Synthase 2 proteinDelta-Ala Synthase 2 proteinDelta-Aminolevulinate Synthase 2 proteinALAS2 proteinALASE proteinASB protein |
Information sourced from Uniprot.org