ALAS1

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.

Porphyrin-containing compound metabolism; protoporphyrin-IX biosynthesis; 5-aminolevulinate from glycine: step 1/1.

In normoxia, is hydroxylated at Pro-576, promoting interaction with VHL, initiating ubiquitination and subsequent degradation via the proteasome.

Ubiquitinated; in normoxia following hydroxylation and interaction with VHL, leading to its subsequent degradation via the proteasome.

Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.

• Mitochondrion inner membrane
• Peripheral membrane protein
• Localizes to the matrix side of the mitochondrion inner membrane.

ALAS3, ALASH, OK/SW-cl.121, ALAS1, ALAS-H, 5-aminolevulinic acid synthase 1, Delta-ALA synthase 1, Delta-aminolevulinate synthase 1

• entrezGene:211
• omim:125290
• swissprot:P13196

Proteins

Metabolism

70581Da