Inhibition of beta-ureidopropionase by propionate may contribute to the neurological complications in patients with propionic acidaemia.
van Gennip
A H
AH
Academic Medical Center, Department of Pediatrics, Amsterdam, The Netherlands.
van Lenthe
H
H
Abeling
N G
NG
Scholten
E G
EG
van Kuilenburg
A B
AB
D000328:Adult; D000581:Amidohydrolases / Q000037:antagonists & inhibitors* / Q000378:metabolism; D002851:Chromatography, High Pressure Liquid; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000235:genetics; D008052:Lipid Metabolism, Inborn Errors / Q000201:enzymology* / Q000235:genetics; D008099:Liver / Q000187:drug effects / Q000201:enzymology; D008297:Male; D009422:Nervous System Diseases / Q000201:enzymology* / Q000235:genetics; D011422:Propionates / Q000097:blood / Q000378:metabolism / Q000494:pharmacology*; D011743:Pyrimidines / Q000378:metabolism
D011422:Propionates; D011743:Pyrimidines; D000581:Amidohydrolases; C021590:beta-ureidopropionase