Propionylcarnitine excretion is not affected by metronidazole administration to patients with disorders of propionate metabolism.
Propionylcarnitine (PC) excretion has been measured during a clinical trial of metronidazole therapy in two patients with propionic acidaemia and two patients with methylmalonic aciduria. All patients were in good metabolic control and were receiving L-carnitine. While total propionate excretion was reduced by up to 40% in all four patients during metronidazole therapy, the excretion of propionylcarnitine remained largely unchanged. PC comprised up to 80% of total propionate excretion in patients with propionic acidaemia.


CONCLUSIONS
These results suggest an extra-hepatic source and/or differing compartmentation for PC formation from those for the production of other metabolites of propionyl-CoA.
Burns
S P
SP
Medical Unit, London Hospital Medical College, UK.
Iles
R A
RA
Saudubray
J M
JM
Chalmers
R A
RA
D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy* / Q000652:urine; D000994:Antitrichomonal Agents / Q000494:pharmacology* / Q000627:therapeutic use; D002331:Carnitine / Q000031:analogs & derivatives* / Q000652:urine; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D009682:Magnetic Resonance Spectroscopy; D008764:Methylmalonic Acid; D008795:Metronidazole / Q000494:pharmacology* / Q000627:therapeutic use; D011422:Propionates
D000994:Antitrichomonal Agents; D011422:Propionates; D008795:Metronidazole; C003223:propionylcarnitine; D008764:Methylmalonic Acid; D002331:Carnitine