KROK 1 Stomatology 2014 – Question 126 · KROK (Dentistry)

Patients with erythropoietic porphyria (Gunther’s disease) have teeth that fluoresce red on exposure to ultraviolet light; photosensitive skin; red urine. This disease is associated with the lack of the following enzyme:

  1. A. Uroporphyrinogen decarboxylase
  2. B. Delta-aminolevulinate synthase
  3. C. Uroporphyrinogen-I synthase
  4. D. Ferrochelatase
  5. E. Uroporphyrinogen-III cosynthase
Hint

Gunther's disease is a congenital erythropoietic porphyria in which type I porphyrins accumulate; think about at which step of haem synthesis the type III isomer is normally formed.

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