title: Carnitine reduces fasting ketogenesis in patients with disorders of propionate metabolism.
abstract: 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.
authors.forename: J A
authors.initials: JA
authors.lastname: Wolff
authors.forename: J E
authors.initials: JE
authors.lastname: Carroll
authors.lastname: Le Phuc Thuy
authors.forename: C
authors.initials: C
authors.lastname: Prodanos
authors.forename: R
authors.initials: R
authors.lastname: Haas
authors.forename: W L
authors.initials: WL
authors.lastname: Nyhan
mesh_terms: 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
chemical_list: D007657:Ketone Bodies; D007659:Ketones; D011422:Propionates; D008764:Methylmalonic Acid; D002331:Carnitine