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doi: 10.1016/j.fct.2023.113726 85 ZouZ.HuW.KangF.XuZ.LiY.ZhangJ.et al

The pathway begins in the mitochondrial matrix with the rate-limiting reaction catalyzed by 5-aminolevulinic acid synthase (ALAS), a PLP-dependent homodimeric enzyme that condenses succinyl-CoA and glycine to produce 5-aminolevulinic acid (ALA), releasing CO and CoASH