COQ2 and multiple system atrophy: As mitochondrial dysfunction is a valid candidate pathogenic mechanism in MSA as suggested by the finding of Coq2 mutations in MSA families (The Multiple-System Atrophy Research Collaboration 2013) and the central role of αSYN in the pathogenesis of MSA is unequivocal, it seems that the combined PLP-αSYN + 3NP model provides a relevant tool to address these two factors, their interplay and interaction with neuroinflammatory responses.