KROK 1 2014 – Question 107 · KROK (Medicine) · Biological Chemistry
Patients with erythropoietic porphyria (Gunther’s disease) have teeth that fluoresce with bright red color when subjected to ultraviolet radiation; their skin is light-sensitive, urine is red-colored. What enzyme can cause this disease, when it is deficient?
- A. Delta-aminolevulinate synthase
- B. Uroporphyrinogen decarboxylase
- C. Uroporphyrinogen I synthase
- D. Ferrochelatase
- E. Uroporphyrinogen III cosynthase
Hint
Link the clinical picture (red urine, fluorescing teeth, skin photosensitivity) with the stage of the porphyrin pathway at which pigments accumulate and with the cell type affected.
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