Loss-of-function-mutations of the chorein encoding gene VPS13A (vacuolar protein sorting-associated protein 13A) lead to chorea-acanthocytosis (ChAc)4, 5, a progressive autosomal recessive neurodegenerative disease with hyperkinetic movements, impaired cognitive functions, myopathy with increased plasma levels of creatine kinase, and erythrocyte acanthocytosis4, 6–8. Here, VPS13A is linked to myopathy.