Costunolide Ameliorates the Methylmalonic Acidemia Via the PINK1/Parkin Pathway.

Methylmalonic acidemia (MMA) is a congenital organic acidemia characterized by mitochondrial dysfunction due to the abnormal accumulation of intermediate metabolites, which subsequently leads to brain damage. Currently, there are no specific pharmacological treatments available for MMA in clinical practice. Costunolide (COS) is a sesquiterpenoid compound derived from Radix Aucklandiae, it exhibits a broad spectrum of bioactivities.