Porphyrin
Biosynthesis
Columns: Enzyme | Location | Substrate | Product | Chromosome | EC | OMIM | Porphyria
ALA synthase | Mitochondrion | Glycine , succinyl CoA | δ-Aminolevulinic acid | 3p21.1 | 2.3.1.37 | 125290 | none | ALA dehydratase | Cytosol | δ-Aminolevulinic acid | Porphobilinogen | 9q34 | 4.2.1.24 | 125270 | ALA-Dehydratase deficiency | PBG deaminase | Cytosol | Porphobilinogen | Hydroxymethyl bilane | 11q23.3 | 2.5.1.61 | 176000 | acute intermittent porphyria | Uroporphyrinogen III synthase | Cytosol | Hydroxymethyl bilane | Uroporphyrinogen III | 10q25.2-q26.3 | 4.2.1.75 | 606938 | congenital erythropoietic porphyria | Uroporphyrinogen III decarboxylase | Cytosol | Uroporphyrinogen III | Coproporphyrinogen III | 1p34 | 4.1.1.37 | 176100 | porphyria cutanea tarda | Coproporphyrinogen III oxidase | Mitochondrion | Coproporphyrinogen III | Protoporphyrinogen IX | 3q12 | 1.3.3.3 | 121300 | coproporphyria | Protoporphyrinogen oxidase | Mitochondrion | Protoporphyrinogen IX | Protoporphyrin IX | 1q22 | 1.3.3.4 | 600923 | variegate porphyria