Determination of urinary orotic acid and uracil by capillary zone electrophoresis.
We describe a simple method for measuring orotic acid and uracil concentration in urine by capillary zone electrophoresis in 20 mM Na-borate buffer, pH 9.2. The method was applied for studying a patient with HHH (hyperomithinemia, hyperammonemia and homocitrullinuria) syndrome. A high value of uracil excretion was found during periods of relatively low orotic acid excretion and normal ammonemia. The orotic acid level in urine was increased by increasing protein intake.
Salerno
C
C
Institute of Paediatrics and Department of Biochemical Sciences, University of Roma La Sapienza, Rome, Italy. ccrifo@axcasp.caspur.it
D'Eufemia
P
P
Celli
M
M
Finocchiaro
R
R
Crifò
C
C
Giardini
O
O
D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood*; D000641:Ammonia / Q000097:blood; D001881:Borates; D002021:Buffers; D002956:Citrulline / Q000031:analogs & derivatives / Q000097:blood; D004044:Dietary Proteins / Q000008:administration & dosage; D019075:Electrophoresis, Capillary / Q000379:methods*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007223:Infant; D008297:Male; D009952:Ornithine / Q000097:blood; D009963:Orotic Acid / Q000652:urine*; D013577:Syndrome; D014498:Uracil / Q000652:urine*
D001881:Borates; D002021:Buffers; D004044:Dietary Proteins; C001352:homocitrulline; D002956:Citrulline; D014498:Uracil; D009963:Orotic Acid; D000641:Ammonia; C010634:sodium borate; D009952:Ornithine