| protoporphyrinogen type III | The immediate precursor of protoporphyrin III in haem biosynthesis; elevated in cases of variegate porphyria. (05 Mar 2000) |
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| protoporphyrinogen type III oxidase | A mitochondrial enzyme that uses O2 to convert protoporphyrinogen type III to protoporphyrin type III in haem biosynthesis; a deficiency of this enzyme is associated with variegate porphyria. (05 Mar 2000) |