Bullet point summary:
What is already known:
The mitochondrial targeting agent, elamipretide, consistently improves pathologic symptoms in subjects with mitochondrial disease.
Elamipretide is safe and well-tolerated; injection site reactions are the most commonly reported adverse event.
What this study adds:
Elamipretide activated MRGPRX2 receptors, suggesting that therapeutically targeting mast cell activation may ameliorate elamipretide ISRs.
Mometasone significantly reduced the incidence of induration/swelling and pruritus following subcutaneous administration of elamipretide.
Clinical Significance:
Mometasone showed promise in mitigating elamipretide-induced injection site reactions and further investigation is warranted.
Treatment options that target mast cell activation may ameliorate elamipretide ISRs and should be explored.
Co-author information: