Marked elevation in plasma trimethylamine-N-oxide (TMAO) in patients with mitochondrial disorders treated with oral l-carnitine.

Molecular Genetics and Metabolism Reports
H D VallanceG Horvath

Abstract

Oral supplementation with l-carnitine is a common therapeutic modality for mitochondrial disorders despite limited evidence of efficacy. Recently, a number of studies have demonstrated that a gut microbiota-dependent metabolite of l-carnitine, trimethylamine oxide (TMAO), is an independent and dose-dependent risk factor for cardiovascular disease (CVD). Given the limited data demonstrating efficacy with oral l-carnitine therapy and the newly raised questions of potential harm, we assessed plasma TMAO levels in patients with mitochondrial disease with and without oral l-carnitine supplementation. Nine subjects were recruited and completed the study. Eight out of 9 subjects at baseline had plasma TMAO concentrations <97.5th percentile (<15.5 μM). One subject with stage 3 renal disease, had marked elevation in plasma TMAO (pre 33.98 μm versus post 101.6 μm). Following at least 3 months of l-carnitine supplementation (1000 mg per day), plasma TMAO levels were markedly increased in 7out of 9 subjects; overall, plasma TMAO significantly increased 11.8-fold (p < 0.001) from a baseline median level of 3.54 μm (interquartile range (IQR) 2.55-8.72) to 43.26 (IQR 23.99-56.04) post supplementation. The results of this study demonstrate tha...Continue Reading

Citations

Jul 29, 2020·Nutrition Research Reviews·Saba NaghipourJohn P Headrick
Sep 26, 2019·Critical Reviews in Food Science and Nutrition·Muthukumaran JayachandranBaojun Xu
Sep 24, 2020·Nature Reviews. Nephrology·Denise MafraPeter Stenvinkel
Oct 23, 2019·Oxidative Medicine and Cellular Longevity·Robert Antoni OlekWieslawa Lysiak-Szydlowska
Sep 11, 2019·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Mohammed AlmannaiAyman W El-Hattab
May 7, 2020·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Alessandra DurazzoAntonello Santini
Sep 23, 2020·Journal of the International Society of Sports Nutrition·Angelika K SawickaRobert A Olek
Jul 17, 2019·Clinical Therapeutics·Heitor O SantosRodrigo C O Macedo
Oct 30, 2020·International Journal of Molecular Sciences·Sophia L SamodelovMichele Visentin
Jun 25, 2021·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Laura BordoniRobert A Olek
Aug 8, 2021·International Journal of Molecular Sciences·Pamela SenesiIleana Terruzzi

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BETA
antisense oligonucleotide

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