Protoporphyrinogen oxidase and ferrochelatase in porphyria variegata.
Protoporphyrinogen oxidase activity and ferrochelatase activity were measured in leucocytes from patients with porphyria variegata. The mean activity of protoporphyrinogen oxidase (PPO) in porphyria variegata (PV) was about 50% of normal (P less than 0.05). The mean activity of ferrochelatase with 59Fe2+ sulphate and protoporphyrin as substrates (in the presence of ascorbic acid) was reduced by 40% (P less than 0.009). The mean activity of ferrochelatase with 59Fe3+ chloride and protoporphyrin as substrates (in the presence of reduced glutathione) was increased by 65% (P less than 0.005). Both are statistically highly significant. The findings are interpreted as follows: (a) The occurrence of a low level of protoporphyrinogen oxidase in PV is confirmed. (b) The findings indicate a concurrent structural change in ferrochelatase (this may be structurally related to (a) but no evidence of this is at present available).
D000328:Adult; D001205:Ascorbic Acid / Q000494:pharmacology; D002712:Chlorides; D005260:Female; D005290:Ferric Compounds / Q000378:metabolism; D005294:Ferrochelatase / Q000097:blood*; D005296:Ferrous Compounds / Q000378:metabolism; D005420:Flavoproteins; D005978:Glutathione / Q000494:pharmacology; D006418:Heme / Q000096:biosynthesis; D006801:Humans; D007962:Leukocytes / Q000201:enzymology*; D008190:Lyases / Q000097:blood*; D008297:Male; D008875:Middle Aged; D024101:Mitochondrial Proteins; D010088:Oxidoreductases / Q000172:deficiency*; D044925:Oxidoreductases Acting on CH-CH Group Donors; D011164:Porphyrias / Q000201:enzymology*; D050768:Protoporphyrinogen Oxidase; D011524:Protoporphyrins / Q000378:metabolism
D002712:Chlorides; D005290:Ferric Compounds; D005296:Ferrous Compounds; D005420:Flavoproteins; D024101:Mitochondrial Proteins; D011524:Protoporphyrins; C020748:ferrous sulfate; D006418:Heme; C028025:protoporphyrin IX; D010088:Oxidoreductases; D044925:Oxidoreductases Acting on CH-CH Group Donors; C493978:PPOX protein, human; D050768:Protoporphyrinogen Oxidase; D008190:Lyases; D005294:Ferrochelatase; D005978:Glutathione; D001205:Ascorbic Acid; C024555:ferric chloride