[Effects of propionic acid metabolic precursors in biotin-deprived rats].
Biotin deficiency induced in the rat, with a biotin-deficient diet but with added avidin involves clinical symptoms of deficiency as well as an important drop (80%) in propionyl-CoA carboxylase activity in the liver, heart and kidneys. But major biochemical anomalies (ketoacidosis and increased urinary elimination of propionic acid), characteristic of propionic acidaemia due to propionyl-CoA carboxylase deficiency in man, are not observed in the rat. Nevertheless, abnormal urinary elimination of methylcitrate and tiglylglycine reflects an appreciable decrease in the metabolism of propionyl-CoA in these animals. The propionyl-CoA load caused by the administration of metabolic precursors of this substance, mainly L-isoleucine, does not induce important biochemical variations except for excretion of propionylglycine.
D000818:Animals; D001360:Avidin / Q000494:pharmacology; D001710:Biotin / Q000172:deficiency*; D002262:Carboxy-Lyases / Q000378:metabolism*; D007532:Isoleucine / Q000494:pharmacology*; D007668:Kidney / Q000201:enzymology; D008099:Liver / Q000201:enzymology; D008297:Male; D043586:Methylmalonyl-CoA Decarboxylase; D009206:Myocardium / Q000201:enzymology; D011422:Propionates / Q000097:blood / Q000378:metabolism / Q000652:urine; D051381:Rats
D011422:Propionates; D007532:Isoleucine; D001360:Avidin; D001710:Biotin; D002262:Carboxy-Lyases; D043586:Methylmalonyl-CoA Decarboxylase
Effets de l'administration de précurseurs métaboliques du propionate à des rats carencés en biotine.