uroporphyrinogen I


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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 sunlight. This is observed in congenital erythropoietic porphyria.