Why the study?
To evaluate the hypothesis that mitochondrial diseases manifest as a nitric oxide-deficiency endotheliopathy and to define vascular impairment and responsiveness to NO-precursor therapy.
Does nitric oxide synthesis-precursor therapy (l-arginine or l-citrulline) improve vascular reactivity, NO signaling, and myocardial bioenergetics in patients with mitochondrial disease?
Comparison
Mitochondrial disease vs controls and NO-precursor therapy vs baseline
Design
Systematic review and quantitative synthesis
Key result
In a systematic review of 157 individuals, mitochondrial disease was associated with marked endothelial impairment that improved following l-arginine or l-citrulline supplementation.
Authors
Loading...
May support nitric oxide precursor trials in mitochondrial disease; extends endothelial dysfunction evidence but leaves clinical adoption open.
Systematic Review (n=157)
Does nitric oxide synthesis-precursor therapy (l-arginine or l-citrulline) improve vascular reactivity, NO signaling, and myocardial bioenergetics in patients with mitochondrial disease?
Mitochondrial disease features a reversible NO-deficiency endotheliopathy that can be partially restored with l-arginine or l-citrulline supplementation, highlighting the vascular endothelium as a potential therapeutic target.
Adams et al. (2026) conducted a systematic review in Mitochondrial disease (n=157). l-arginine or l-citrulline supplementation vs. Controls or baseline was evaluated on Vascular reactivity, biochemical NO production, and myocardial metabolic imaging. In a systematic review of 157 individuals, mitochondrial disease was associated with marked endothelial impairment that improved following l-arginine or l-citrulline supplementation.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: