[Porphobilinogen synthase activity and porphyrin biosynthesis in the erythrocytes after incubation with delta-aminolevulinic acid in pernicious anemia patients].
The activity of PBG-S was studied in erythrocytes according the standard method of Berlin and Schaller as well as the biosynthesis of porphyrins after incubation with delta-aminolevulinic acid according to Idel'son in a control group of 40 healthy males and females and 15 patients with pernicious anemia. The activity of porphobilinogen synthase and the level of porphobilinogen were elevated in the patients studied. The total quantity of synthesized porphyrins is increased as regards the control group of healthy on the account of the enhanced biosynthesis of uroporphyrins and coproporphyrins, whereas the quantity of the protoporphyrin synthesized was reduced. The changes established reflect the secondary disordered heme synthesis in the patients with pernicious anemia.
D000622:Aminolevulinic Acid / Q000494:pharmacology*; D000752:Anemia, Pernicious / Q000097:blood*; D004912:Erythrocytes / Q000187:drug effects / Q000378:metabolism*; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007982:Levulinic Acids / Q000494:pharmacology*; D008297:Male; D000623:Porphobilinogen Synthase / Q000097:blood*; D011166:Porphyrins / Q000096:biosynthesis*
D006454:Hemoglobins; D007982:Levulinic Acids; D011166:Porphyrins; D000622:Aminolevulinic Acid; D000623:Porphobilinogen Synthase
Porfobilinogensintazna aktivnost i biosinteza na porfirinite v eritrotsitite sled induktsiia s delta-aminolevulinova kiselina pri bolni s pernitsiozna anemiia.