Endothelial Dysfunction and the Effect of Arginine and Citrulline Supplementation in Children and Adolescents With Mitochondrial Diseases
Background: In addition to the reduced energy production, characteristic of mitochondrial disorders, nitric oxide (NO) deficiency can occur as well. The NO produced by vascular endothelial cells relaxes vascular smooth muscles, resulting in vasodilation that maintains the patency of small blood vess...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
SAGE Publishing
2020-02-01
|
| Series: | Journal of Central Nervous System Disease |
| Online Access: | https://doi.org/10.1177/1179573520909377 |
| _version_ | 1818054919866810368 |
|---|---|
| author | Fatma Al Jasmi Nuha Al Zaabi Khalid Al-Thihli Amal M Al Teneiji Jozef Hertecant Ayman W El-Hattab |
| author_facet | Fatma Al Jasmi Nuha Al Zaabi Khalid Al-Thihli Amal M Al Teneiji Jozef Hertecant Ayman W El-Hattab |
| author_sort | Fatma Al Jasmi |
| collection | DOAJ |
| description | Background: In addition to the reduced energy production, characteristic of mitochondrial disorders, nitric oxide (NO) deficiency can occur as well. The NO produced by vascular endothelial cells relaxes vascular smooth muscles, resulting in vasodilation that maintains the patency of small blood vessels and promotes blood flow through microvasculature. Endothelial dysfunction due to inability of vascular endothelium to generate enough NO to maintain adequate vasodilation can result in decreased perfusion in the microvasculature of various tissues, contributing to many complications seen in individuals with mitochondrial diseases. The amino acids arginine and citrulline are NO precursors: increasing their concentrations could potentially restore NO production. Methods: In this study, we assessed endothelial dysfunction in children and adolescents with mitochondrial diseases. We also investigated the effect of arginine and citrulline supplementation on endothelial dysfunction in these individuals. We used peripheral arterial tonometry to measure the reactive hyperemic index (RHI), which is low when there is endothelial dysfunction. Results: The results demonstrated low RHI in individuals with mitochondrial diseases, indicating endothelial dysfunction. RHI increased with arginine or citrulline supplementation suggesting that supplementation with NO precursors can improve endothelial dysfunction by enhancing NO production. Conclusions: This study is the first one to use peripheral arterial tonometry methodology in mitochondrial diseases. The results of this study provide evidence for endothelial dysfunction in mitochondrial diseases and demonstrate that arginine or citrulline supplementation can alleviate the endothelial dysfunction, providing more evidence for the potential therapeutic utility of these amino acids in mitochondrial diseases. |
| first_indexed | 2024-12-10T12:04:42Z |
| format | Article |
| id | doaj.art-61e111bc78bd4fd5b274a8c3c311dfeb |
| institution | Directory Open Access Journal |
| issn | 1179-5735 |
| language | English |
| last_indexed | 2024-12-10T12:04:42Z |
| publishDate | 2020-02-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Journal of Central Nervous System Disease |
| spelling | doaj.art-61e111bc78bd4fd5b274a8c3c311dfeb2022-12-22T01:49:30ZengSAGE PublishingJournal of Central Nervous System Disease1179-57352020-02-011210.1177/1179573520909377Endothelial Dysfunction and the Effect of Arginine and Citrulline Supplementation in Children and Adolescents With Mitochondrial DiseasesFatma Al Jasmi0Nuha Al Zaabi1Khalid Al-Thihli2Amal M Al Teneiji3Jozef Hertecant4Ayman W El-Hattab5Department of Pediatrics, College of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab EmiratesDepartment of Pediatrics, College of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab EmiratesGenetic and Developmental Medicine Clinic, Sultan Qaboos University Hospital, Muscat, OmanGenetic and Metabolic Division, Pediatrics Department, Sheikh Khalifa Medical City, Abu Dhabi, United Arab EmiratesDivision of Clinical Genetic and Metabolic Disorders, Tawam Hospital, Al-Ain, United Arab EmiratesDepartment of Clinical Sciences, College of Medicine, University of Sharjah, Sharjah, United Arab EmiratesBackground: In addition to the reduced energy production, characteristic of mitochondrial disorders, nitric oxide (NO) deficiency can occur as well. The NO produced by vascular endothelial cells relaxes vascular smooth muscles, resulting in vasodilation that maintains the patency of small blood vessels and promotes blood flow through microvasculature. Endothelial dysfunction due to inability of vascular endothelium to generate enough NO to maintain adequate vasodilation can result in decreased perfusion in the microvasculature of various tissues, contributing to many complications seen in individuals with mitochondrial diseases. The amino acids arginine and citrulline are NO precursors: increasing their concentrations could potentially restore NO production. Methods: In this study, we assessed endothelial dysfunction in children and adolescents with mitochondrial diseases. We also investigated the effect of arginine and citrulline supplementation on endothelial dysfunction in these individuals. We used peripheral arterial tonometry to measure the reactive hyperemic index (RHI), which is low when there is endothelial dysfunction. Results: The results demonstrated low RHI in individuals with mitochondrial diseases, indicating endothelial dysfunction. RHI increased with arginine or citrulline supplementation suggesting that supplementation with NO precursors can improve endothelial dysfunction by enhancing NO production. Conclusions: This study is the first one to use peripheral arterial tonometry methodology in mitochondrial diseases. The results of this study provide evidence for endothelial dysfunction in mitochondrial diseases and demonstrate that arginine or citrulline supplementation can alleviate the endothelial dysfunction, providing more evidence for the potential therapeutic utility of these amino acids in mitochondrial diseases.https://doi.org/10.1177/1179573520909377 |
| spellingShingle | Fatma Al Jasmi Nuha Al Zaabi Khalid Al-Thihli Amal M Al Teneiji Jozef Hertecant Ayman W El-Hattab Endothelial Dysfunction and the Effect of Arginine and Citrulline Supplementation in Children and Adolescents With Mitochondrial Diseases Journal of Central Nervous System Disease |
| title | Endothelial Dysfunction and the Effect of Arginine and Citrulline Supplementation in Children and Adolescents With Mitochondrial Diseases |
| title_full | Endothelial Dysfunction and the Effect of Arginine and Citrulline Supplementation in Children and Adolescents With Mitochondrial Diseases |
| title_fullStr | Endothelial Dysfunction and the Effect of Arginine and Citrulline Supplementation in Children and Adolescents With Mitochondrial Diseases |
| title_full_unstemmed | Endothelial Dysfunction and the Effect of Arginine and Citrulline Supplementation in Children and Adolescents With Mitochondrial Diseases |
| title_short | Endothelial Dysfunction and the Effect of Arginine and Citrulline Supplementation in Children and Adolescents With Mitochondrial Diseases |
| title_sort | endothelial dysfunction and the effect of arginine and citrulline supplementation in children and adolescents with mitochondrial diseases |
| url | https://doi.org/10.1177/1179573520909377 |
| work_keys_str_mv | AT fatmaaljasmi endothelialdysfunctionandtheeffectofarginineandcitrullinesupplementationinchildrenandadolescentswithmitochondrialdiseases AT nuhaalzaabi endothelialdysfunctionandtheeffectofarginineandcitrullinesupplementationinchildrenandadolescentswithmitochondrialdiseases AT khalidalthihli endothelialdysfunctionandtheeffectofarginineandcitrullinesupplementationinchildrenandadolescentswithmitochondrialdiseases AT amalmalteneiji endothelialdysfunctionandtheeffectofarginineandcitrullinesupplementationinchildrenandadolescentswithmitochondrialdiseases AT jozefhertecant endothelialdysfunctionandtheeffectofarginineandcitrullinesupplementationinchildrenandadolescentswithmitochondrialdiseases AT aymanwelhattab endothelialdysfunctionandtheeffectofarginineandcitrullinesupplementationinchildrenandadolescentswithmitochondrialdiseases |