Mitochondria: Role of citrulline and arginine supplementation in MELAS syndrome.

Mitochondria: Role of citrulline and arginine supplementation in MELAS syndrome.
Int J Biochem Cell Biol. 2014 Jan 8;
Authors: El-Hattab AW, Emrick LT, Chanprasert S, Craigen WJ, Scaglia F
Abstract
Mitochondria are found in all nucleated human cells and generate most of the cellular energy. Mitochondrial disorders result from dysfunctional mitochondria that are unable to generate sufficient ATP to meet the energy needs of various organs. Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a frequent maternally inherited mitochondrial disorder. There is growing evidence that nitric oxide (NO) deficiency occurs in MELAS syndrome and results in impaired blood perfusion that contributes significantly to several complications including stroke-like episodes, myopathy, and lactic acidosis. Both arginine and citrulline act as NO precursors and their administration results in increased NO production and hence can potentially have therapeutic utility in MELAS syndrome. Citrulline raises NO production to a greater extent than arginine, therefore, citrulline may have a better therapeutic effect. Controlled studies assessing the effects of arginine or citrulline supplementation on different clinical aspects of MELAS syndrome are needed.
PMID: 24412347 [PubMed - as supplied by publisher]

Source: The International Journal of Biochemistry and Cell Biology - January 8, 2014 Category: Biochemistry Authors: El-Hattab AW, Emrick LT, Chanprasert S, Craigen WJ, Scaglia F Tags: Int J Biochem Cell Biol Source Type: research