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 urin...

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Bibliographic Details
Published in:Nutrition and metabolism (Basel) 1980, Vol.24 (6), p.367-382
Main Authors: Cherruau, B, Mangeot, M, Demelier, J F, Charpentier, C, Pelletier, C, Lemonnier, A
Format: Article
Language:fre
Subjects:
Animals
Avidin - pharmacology
Biotin - deficiency
Carboxy-Lyases - metabolism
Isoleucine - pharmacology
Kidney - enzymology
Liver - enzymology
Male
Methylmalonyl-CoA Decarboxylase
Myocardium - enzymology
Propionates - blood
Propionates - metabolism
Propionates - urine
Rats
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Summary: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.
ISSN:0029-6678