Protoporphyrinogen oxidase and porphobilinogen deaminase in variegate porphyria.

  title={Protoporphyrinogen oxidase and porphobilinogen deaminase in variegate porphyria.},
  author={Peter Mei\ssner and Rufus S. Day and Michael R. Moore and P B Disler and Eric Harley},
  journal={European journal of clinical investigation},
  volume={16 3},
Two enzymes of the haem biosynthetic pathway were investigated in patients with variegate porphyria. Protoporphyrinogen oxidase in cultures of Epstein-Barr virus transformed lymphoblasts from twenty-seven patients showed a mean maximal velocity (Vmax) of 0.39 +/- 0.08+ nmol of protoporphyrin mg protein-1 h-1, a 52% reduction (P less than 0.001) from a non-porphyric control group (0.82 +/- 0.10). Km values (1.00 +/- 0.27 microM) did not differ significantly (P greater than 0.05) from control… CONTINUE READING


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