Arginine and Citrulline for the Treatment of MELAS Syndrome
AUTOR(ES)
El-Hattab, Ayman W.
FONTE
J. inborn errors metab. screen.
DATA DE PUBLICAÇÃO
16/05/2019
RESUMO
Abstract Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disease with a broad spectrum of manifestations. In addition to impaired energy production, nitric oxide (NO) deficiency occurs in MELAS syndrome and leads to impaired blood perfusion in microvasculature that can contribute to several complications including stroke-like episodes, myopathy, and lactic acidosis. The supplementation of NO precursors, L-arginine and L-citrulline, increases NO production and hence can potentially have therapeutic utility in MELAS syndrome. L-citrulline raises NO production to a greater extent than L-arginine; therefore, L-citrulline may have a better therapeutic effect. The clinical effect of L-citrulline has not yet been studied and clinical studies on L-arginine, which are limited, only evaluated the stroke-like episodes’ aspects of the disease. Controlled studies are still needed to assess the clinical effects of L-arginine and L-citrulline on different aspects of MELAS syndrome.
Documentos Relacionados
- Plasma arginine and citrulline kinetics in adults given adequate and arginine-free diets.
- Arabidopsis chloroplasts dissimilate L-arginine and L-citrulline for use as N source.
- Arginine biosynthesis in Neisseria gonorrhoeae: enzymes catalyzing the formation of ornithine and citrulline.
- Ornithine, Citrulline, and Arginine Metabolism in Watermelon Seedlings. 1
- The metabolism of L-arginine and its significance for the biosynthesis of endothelium-derived relaxing factor: cultured endothelial cells recycle L-citrulline to L-arginine.