Effect of haem arginate therapy on porphyrin metabolism and mixed function oxygenase activity in acute hepatic porphyria.
The effect of haem arginate on porphyrin metabolism and haemoprotein function was studied during seven attacks of acute hepatic porphyria in 5 patients. In each attack it greatly reduced the overproduction of porphyrin precursors and repressed the overactivity of the rate-controlling enzyme of haem synthesis delta-aminolaevulinic acid (ALA) synthase measured in leucocytes. Antipyrine clearance, an index of the oxidative function of cytochromes P-450, the major group of hepatic haemoproteins, was increased during haem therapy. Thus, haem arginate not only suppresses the overproduction of haem precursors but also improves hepatic oxidative metabolism in acute porphyria.
D000208:Acute Disease; D000328:Adult; D000983:Antipyrine / Q000378:metabolism; D001120:Arginine / Q000494:pharmacology / Q000627:therapeutic use*; D003577:Cytochrome P-450 Enzyme System / Q000378:metabolism; D003864:Depression, Chemical; D005260:Female; D006418:Heme / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D011163:Hydroxymethylbilane Synthase / Q000378:metabolism; D007962:Leukocytes / Q000201:enzymology; D008099:Liver / Q000378:metabolism; D008107:Liver Diseases / Q000188:drug therapy* / Q000378:metabolism; D008297:Male; D008930:Mitochondria, Liver / Q000378:metabolism; D006899:Mixed Function Oxygenases / Q000378:metabolism*; D000623:Porphobilinogen Synthase / Q000378:metabolism; D011164:Porphyrias / Q000188:drug therapy* / Q000378:metabolism; D011166:Porphyrins / Q000378:metabolism*
D011166:Porphyrins; D006418:Heme; D003577:Cytochrome P-450 Enzyme System; D001120:Arginine; D006899:Mixed Function Oxygenases; D011163:Hydroxymethylbilane Synthase; D000623:Porphobilinogen Synthase; C048849:heme arginate; D000983:Antipyrine