Heme biosynthetic pathway enzyme (None standardized for the entire pathway; most enzymes in the pathway have individual abbreviations (e.g., ALAS for 5-aminolevulinic acid synthase, PBGD for porphobilinogen deaminase))

The heme biosynthetic pathway consists of a series of eight sequential enzymatic steps converting glycine and succinyl-CoA into heme through multiple intermediates (ALA, PBG, HMB, uroporphyrinogen III, coproporphyrinogen III, protoporphyrinogen IX, protoporphyrin IX), with each reaction catalyzed by a specific enzyme (e.g., ALAS, ALAD, PBGD, UROS, UROD, CPO, PPO, FC). These enzymes are critical in cellular metabolism and function of hemoglobin, cytochromes, and other heme-containing proteins. Mutations or inhibition of these enzymes lead to a variety of porphyrias and other metabolic disorders, making them both biomarkers and therapeutic targets. The canonical nomenclature for each enzyme should be used specifically, rather than the collective term. If a database entry requires a single canonical molecule, split this entry into individual enzyme names for accuracy and utility.

Other names

Heme biosynthetic enzymesHeme synthesis enzymesPorphyrin pathway enzymesIndividual enzyme names as aliases: 5-aminolevulinic acid synthase (ALAS)porphobilinogen deaminase (PBGD)uroporphyrinogen III synthase (UROS)uroporphyrinogen III decarboxylase (UROD)coproporphyrinogen III oxidase (CPO)protoporphyrinogen IX oxidase (PPO)ferrochelatase (FC)etc.

Enzyme inhibition (e.g., reducing ALAS1 activity); Enzyme replacement or activation; Gene silencing via RNA interference (e.g., givosiran targets ALAS1 mRNA)