Nuclear gene variants associated with canine mitochondrial myopathies include a mutation in the PDP1 gene (OMIA#001406-9615), encoding the phosphatase enzyme that activates the pyruvate dehydrogenase complex, in Clumber and Sussex spaniels [20]; a variant in the mitochondrial aspartate/glutamate carrier gene SLC25A12 (OMIA#002294-9615) that causes an inflammatory myopathy in Dutch shepherd dogs [21]; and a variant in the ACADVL gene (OMIA# 002140-9615) encoding the very-long-chain acryl-CoA dehydrogenase associated with exercise-induced myopathy in German Hunting terriers [22]. This evidence concerns the gene ACADVL and myopathy.