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.
- Treatment options that target mast cell activation may ameliorate
elamipretide ISRs and should be explored.