Title |
Arginine and Citrulline for the Treatment of MELAS Syndrome
|
---|---|
Published in |
Journal of Inborn Errors of Metabolism and Screening, March 2017
|
DOI | 10.1177/2326409817697399 |
Pubmed ID | |
Authors |
Ayman W. El-Hattab, Mohammed Almannai, Fernando Scaglia |
Abstract |
MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) 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 aspect 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. |
X Demographics
Geographical breakdown
Country | Count | As % |
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United States | 3 | 60% |
Unknown | 2 | 40% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 4 | 80% |
Science communicators (journalists, bloggers, editors) | 1 | 20% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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Unknown | 59 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Bachelor | 10 | 17% |
Researcher | 7 | 12% |
Other | 5 | 8% |
Student > Ph. D. Student | 5 | 8% |
Student > Master | 4 | 7% |
Other | 10 | 17% |
Unknown | 18 | 31% |
Readers by discipline | Count | As % |
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Biochemistry, Genetics and Molecular Biology | 10 | 17% |
Neuroscience | 8 | 14% |
Medicine and Dentistry | 8 | 14% |
Pharmacology, Toxicology and Pharmaceutical Science | 4 | 7% |
Agricultural and Biological Sciences | 2 | 3% |
Other | 8 | 14% |
Unknown | 19 | 32% |