These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
2. Carnitine reduces fasting ketogenesis in patients with disorders of propionate metabolism. Wolff JA; Carroll JE; Le Phuc Thuy ; Prodanos C; Haas R; Nyhan WL Lancet; 1986 Feb; 1(8476):289-91. PubMed ID: 2868163 [TBL] [Abstract][Full Text] [Related]
3. L-carnitine in inborn errors of metabolism: what is the evidence? Walter JH J Inherit Metab Dis; 2003; 26(2-3):181-8. PubMed ID: 12889659 [TBL] [Abstract][Full Text] [Related]
4. [A case of cobalamin-dependent methylmalonic acidemia. Biochemical aspects, diagnosis and possible treatment (author's transl)]. Schuchmann L; Leupold D; Lehnert W Monatsschr Kinderheilkd (1902); 1978 Apr; 126(4):179-83. PubMed ID: 651892 [TBL] [Abstract][Full Text] [Related]
5. Sources of propionate in inborn errors of propionate metabolism. Thompson GN; Walter JH; Bresson JL; Ford GC; Lyonnet SL; Chalmers RA; Saudubray JM; Leonard JV; Halliday D Metabolism; 1990 Nov; 39(11):1133-7. PubMed ID: 2233273 [TBL] [Abstract][Full Text] [Related]
6. Inborn errors of propionate metabolism: methylmalonic and propionic acidaemias. Thompson GN J Paediatr Child Health; 1992 Apr; 28(2):134-5. PubMed ID: 1562361 [No Abstract] [Full Text] [Related]
7. The management and outcome of propionic and methylmalonic acidaemia. Leonard JV J Inherit Metab Dis; 1995; 18(4):430-4. PubMed ID: 7494401 [TBL] [Abstract][Full Text] [Related]
8. Identification of undescribed medium-chain acylcarnitines present in urine of patients with propionic and methylmalonic acidemias. Libert R; Van Hoof F; Thillaye M; Vincent MF; Nassogne MC; de Hoffmann E; Schanck A Clin Chim Acta; 2000 May; 295(1-2):87-96. PubMed ID: 10767396 [TBL] [Abstract][Full Text] [Related]
9. [Studies on the beneficial effect of levocarnitine chloride (LC-80) on organic acidemias, especially propionic acidemia and methylmalonic acidemia]. Fujisawa S; Shimatani K; Yamada H; Hironaka Y Nihon Yakurigaku Zasshi; 1989 May; 93(5):305-14. PubMed ID: 2744657 [TBL] [Abstract][Full Text] [Related]
10. Oxidative stress parameters in urine from patients with disorders of propionate metabolism: a beneficial effect of L:-carnitine supplementation. Ribas GS; Biancini GB; Mescka C; Wayhs CY; Sitta A; Wajner M; Vargas CR Cell Mol Neurobiol; 2012 Jan; 32(1):77-82. PubMed ID: 21833551 [TBL] [Abstract][Full Text] [Related]
11. Metabolism of 1-13C-propionate in vivo in patients with disorders of propionate metabolism. Barshop BA; Yoshida I; Ajami A; Sweetman L; Wolff JA; Sweetman FR; Prodanos C; Smith M; Nyhan WL Pediatr Res; 1991 Jul; 30(1):15-22. PubMed ID: 1909779 [TBL] [Abstract][Full Text] [Related]
12. Urinary propionylcarnitine analysis for monitoring carnitine supplementation in inherited disorders of propionate metabolism. Sugiyama N; Matsuda I; Wada Y; Narisawa K; Kobayashi M; Kidouchi K J Inherit Metab Dis; 1994; 17(5):611-5. PubMed ID: 7837769 [No Abstract] [Full Text] [Related]
13. Stimulation of ketogenesis by propionate in isolated rat hepatocytes: an explanation for ketosis associated with propionic acidaemia and methylmalonic acidaemia? Rosario P; Medina JM J Inherit Metab Dis; 1982; 5(1):59-62. PubMed ID: 6820416 [TBL] [Abstract][Full Text] [Related]
14. Increased urinary metabolite excretion during fasting in disorders of propionate metabolism. Thompson GN; Chalmers RA Pediatr Res; 1990 Apr; 27(4 Pt 1):413-6. PubMed ID: 2342832 [TBL] [Abstract][Full Text] [Related]
15. Rapid diagnosis of methylmalonic and propionic acidemias: quantitative tandem mass spectrometric analysis of propionylcarnitine in filter-paper blood specimens obtained from newborns. Chace DH; DiPerna JC; Kalas TA; Johnson RW; Naylor EW Clin Chem; 2001 Nov; 47(11):2040-4. PubMed ID: 11673377 [No Abstract] [Full Text] [Related]
16. The use of metronidazole in management of methylmalonic and propionic acidaemias. Thompson GN; Chalmers RA; Walter JH; Bresson JL; Lyonnet SL; Reed PJ; Saudubray JM; Leonard JV; Halliday D Eur J Pediatr; 1990 Aug; 149(11):792-6. PubMed ID: 2226555 [TBL] [Abstract][Full Text] [Related]
17. Carnitine therapy and metabolism in the disorders of propionyl-CoA metabolism studied using 1H-NMR spectroscopy. Davies SE; Iles RA; Stacey TE; de Sousa C; Chalmers RA Clin Chim Acta; 1991 Dec; 204(1-3):263-77. PubMed ID: 1819469 [TBL] [Abstract][Full Text] [Related]
18. Contribution of odd-numbered fatty acid oxidation to propionate production in neonates with methylmalonic and propionic acidaemias. Wendel U; Zass R; Leupold D Eur J Pediatr; 1993 Dec; 152(12):1021-3. PubMed ID: 8131803 [TBL] [Abstract][Full Text] [Related]
19. Propionate metabolism by cultured skin fibroblasts from normal individuals and patients with methylmalonicaciduria and propionicacidemia. Oizumi J; Giudici TA; Ng WG; Shaw KN; Donnell GN Biochem Med; 1981 Aug; 26(1):28-40. PubMed ID: 7295302 [No Abstract] [Full Text] [Related]
20. In vivo propionate oxidation as a prognostic indicator in disorders of propionate metabolism. Thompson GN; Walter JH; Bresson JL; Bonnefont JP; Saudubray JM; Leonard JV; Halliday D Eur J Pediatr; 1990 Mar; 149(6):408-11. PubMed ID: 2332010 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]