Clinical Trials Logo

Clinical Trial Summary

The purpose of this study is to assesses the clinical efficacy of oral supplementation with glutamine over 3 years.


Clinical Trial Description

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a genetically heterogeneous disorder. The most common mutation is in the mtDNA gene MT-TL1 encoding the mitochondrial tRNALeu (UUR). For understanding the development of seizures in patients with mitochondrial disease, a study has recently emphasized the deficiency of astrocytic glutamine synthetase, creating a disinhibited neuronal network for seizure generation. The investigators propose to evaluate nine patients with mitochondrial DNA mutation and MELAS. Patients will receive oral supplementation with 12-18 g/day of glutamine (adjusted for weight and plasma concentrations). The primary outcome measures modification in clinical scales. ;


Study Design


Related Conditions & MeSH terms


NCT number NCT05255328
Study type Interventional
Source Hospital Universitario 12 de Octubre
Contact
Status Active, not recruiting
Phase N/A
Start date July 1, 2021
Completion date July 24, 2024

See also
  Status Clinical Trial Phase
Completed NCT01831934 - Responses to Influenza Vaccine in Patients With Mitochondrial Disorders (MELAS) Phase 4
Not yet recruiting NCT06013397 - Effectiveness of Ketogenic Diet in MELAS Syndrome N/A
Completed NCT04165239 - The KHENERGYZE Study Phase 2
Completed NCT01603446 - L-arginine Therapy on Endothelium-dependent Vasodilation & Mitochondrial Metabolism in MELAS Syndrome Phase 2
Completed NCT00887562 - Study of Idebenone in the Treatment of Mitochondrial Encephalopathy Lactic Acidosis & Stroke-like Episodes Phase 2
Active, not recruiting NCT00068913 - Evaluating the Effectiveness of a Dichloroacetate in MELAS Syndrome Phase 2
Completed NCT03888716 - A Phase Ia/Ib, SAD and MAD Study of of KL1333 in Healthy Subjects and Patients With Primary Mitochondrial Disease Phase 1
Completed NCT04948138 - Glutamine Supplement in MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes) Syndrome N/A
Completed NCT03056209 - Safety, Tolerability and Pharmacokinetic Study of KL1333 in Healthy Male Volunteers Phase 1
Completed NCT01339494 - Nitric Oxide Production in MELAS Syndrome Phase 0
Recruiting NCT02114554 - Mitochondrial nt3243 A>G Mutation in Taiwan N/A
Recruiting NCT05554835 - Global Registry and Natural History Study for Mitochondrial Disorders
Completed NCT01252979 - Ketones & Mitochondrial Heteroplasmy Phase 0
Completed NCT00004353 - Study of the Metabolism of Pyruvate and Related Problems in Patients With Lactic Acidemia N/A
Recruiting NCT03952234 - L-Citrulline Dose Finding Safety Study in MELAS Phase 1