| uroporphyrinogen-III synthase |
[EC 4.2.1.75] an enzyme of the lyase class that acts concertedly with hydroxymethylbilane synthase to convert porphobilinogen to uroporphyrinogen III in the biosynthesis of porphyrins and heme. Deficiency of the enzyme, an autosomal recessive trait, causes congenital erythropoietic porphyria. Called also uroporphyrinogen III cosynthase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| uroporphyrinogen I |
An abnormal isomer of a precursor of protoporphyrin, which accumulates in one form of porphyria. It causes the urine to be red, the teeth to fluoresce brightly in ultraviolet light, and the skin to be abnormally sensitive to s
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