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Published on: February 25, 2016
Endothelial dysfunction in MELAS improved by l-arginine supplementation
1Department of Pediatrics and Child Health, Kurume University School of Medicine, Fukuoka, Japan. yasukoga@med.kurume-u.ac.jp
Neurology
|June 14, 2006
Summary
Oral l-arginine supplementation improved endothelial function in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke (MELAS). This treatment normalized endothelial function and showed promise for managing MELAS-related strokelike episodes.
Area of Science:
- Neurology
- Vascular Biology
- Mitochondrial Diseases
Background:
- Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke (MELAS) is a rare genetic disorder.
- Endothelial dysfunction is a key feature in MELAS patients, contributing to strokelike episodes.
- Nitric oxide (NO) plays a crucial role in maintaining endothelial function.
Purpose of the Study:
- To evaluate endothelial function in MELAS patients using flow-mediated dilation (FMD).
- To assess the therapeutic effect of oral l-arginine supplementation on endothelial function in MELAS.
- To investigate the correlation between plasma l-arginine levels and endothelial function improvement.
Main Methods:
- Flow-mediated dilation (FMD) was used to assess endothelial function in MELAS patients and healthy controls.
- Patients received oral l-arginine supplementation for two years.
- Plasma l-arginine levels were measured before and after supplementation.
Main Results:
- MELAS patients exhibited significantly impaired endothelial function compared to controls.
- Two years of l-arginine supplementation significantly improved FMD in MELAS patients, reaching control levels.
- Plasma l-arginine levels were normalized following supplementation, correlating with improved endothelial function.
Conclusions:
- Endothelial dysfunction is prevalent in MELAS and can be improved with l-arginine therapy.
- Oral l-arginine supplementation shows potential as a treatment strategy for MELAS, particularly for reducing strokelike episodes.
- Restoring nitric oxide bioavailability through l-arginine may be a key mechanism in improving MELAS outcomes.

