Simultaneous determination of hydroxymethylbilane synthase and uroporphyrinogen III synthase in erythrocytes by high-performance liquid chromatography.
A high-performance-liquid-chromatographic method is developed for the simultaneous determination of hydroxymethylbilane synthase and uroporphyrinogen III synthase activity in erythrocytes. Effective separation of uroporphyrin I and III isomers allows the accurate quantification of individual isomers and the total uroporphyrin concentration. Total uroporphyrin production is used to calculate hydroxymethylbilane synthase activity, and the amount of uroporphyrin III formed represents the activity of uroporphyrinogen III synthase. Normal ranges are established for the two enzymes.
D000642:Ammonia-Lyases / Q000097:blood*; D002851:Chromatography, High Pressure Liquid / Q000379:methods*; D004912:Erythrocytes / Q000201:enzymology*; D006801:Humans; D011163:Hydroxymethylbilane Synthase / Q000097:blood*; D007535:Isomerases / Q000097:blood*; D011164:Porphyrias / Q000097:blood / Q000201:enzymology; D014576:Uroporphyrinogen III Synthetase / Q000097:blood*
D011163:Hydroxymethylbilane Synthase; D014576:Uroporphyrinogen III Synthetase; D000642:Ammonia-Lyases; D007535:Isomerases