High-performance liquid chromatographic detection of pitfalls in porphobilinogen deaminase determination.
Potential pitfalls in the determination of porphobilinogen deaminase activity, as well as ways of eliminating these sources of error and determining the activity accurately, are discussed. In addition to measurement of the accurate activity, the described method (a combination of incubation of homogenate with porphobilinogen and high-performance liquid chromatographic separation) can also be used to detect enzymic defects in the haem biosynthetic pathway, according to the pattern of accumulation of the various porphyrins.
Schoenfeld
N
N
Laboratory of Biochemical Pharmacology, Beilinson Medical Center, Petah Tiqva, Israel.
Mamet
R
R
D002851:Chromatography, High Pressure Liquid; D004121:Dimethyl Sulfoxide / Q000737:chemistry; D006801:Humans; D011163:Hydroxymethylbilane Synthase / Q000097:blood*; D011166:Porphyrins / Q000096:biosynthesis; D011524:Protoporphyrins / Q000097:blood; D012016:Reference Values; D013050:Spectrometry, Fluorescence; D014238:Trichloroacetic Acid / Q000737:chemistry; D014578:Uroporphyrins / Q000097:blood
D011166:Porphyrins; D011524:Protoporphyrins; D014578:Uroporphyrins; D014238:Trichloroacetic Acid; D011163:Hydroxymethylbilane Synthase; D004121:Dimethyl Sulfoxide