Porphyrin
Biosynthesis
Columns: Enzyme | Location | Substrate | Product | Chromosome | OMIM | Porphyria
ALA synthase | Mitochondrion | Glycine , succinyl CoA | δ-Aminolevulinic acid | 3p21.1 | 125290 | none | ALA dehydratase | Cytosol | δ-Aminolevulinic acid | Porphobilinogen | 9q34 | 125270 | ALA-Dehydratase deficiency | PBG deaminase | Cytosol | Porphobilinogen | Hydroxymethyl bilane | 11q23.3 | 176000 | acute intermittent porphyria | Uroporphyrinogen III synthase | Cytosol | Hydroxymethyl bilane | Uroporphyrinogen III | 10q25.2-q26.3 | 606938 | congenital erythropoietic porphyria | Uroporphyrinogen III decarboxylase | Cytosol | Uroporphyrinogen III | Coproporphyrinogen III | 1p34 | 176100 | porphyria cutanea tarda | Coproporphyrinogen III oxidase | Mitochondrion | Coproporphyrinogen III | Protoporphyrinogen IX | 3q12 | 121300 | coproporphyria | Protoporphyrinogen oxidase | Mitochondrion | Protoporphyrinogen IX | Protoporphyrin IX | 1q22 | 600923 | variegate porphyria | Ferrochelatase | Mitochondrion | Protoporphyrin IX | Heme | 18q21.3 | 177000 | erythropoietic protoporphyria