Carnitine reduces fasting ketogenesis in patients with disorders of propionate metabolism.
Patients with disorders of propionate metabolism have low plasma levels of free carnitine and excrete higher than normal quantities of esterified carnitine. The response to a 19 h fast was assessed as a physiological index of carnitine deficiency. In patients with propionic acidaemia and methylmalonic acidaemia a substantial ketogenesis developed in response to fasting. Supplementation with L-carnitine significantly reduced this ketogenic response.
Wolff
J A
JA
Carroll
J E
JE
Le Phuc Thuy
Prodanos
C
C
Haas
R
R
Nyhan
W L
WL
D002331:Carnitine / Q000097:blood / Q000627:therapeutic use* / Q000652:urine; D002648:Child; D002675:Child, Preschool; D003864:Depression, Chemical; D005215:Fasting; D005260:Female; D006801:Humans; D007223:Infant; D007657:Ketone Bodies / Q000096:biosynthesis*; D007659:Ketones / Q000097:blood; D008297:Male; D008661:Metabolism, Inborn Errors / Q000188:drug therapy*; D008764:Methylmalonic Acid / Q000378:metabolism; D011422:Propionates / Q000378:metabolism*; D013997:Time Factors
D007657:Ketone Bodies; D007659:Ketones; D011422:Propionates; D008764:Methylmalonic Acid; D002331:Carnitine