CPT2 and myopathy: Of note, in another study in mTOR knockout mice presenting with progressive myopathy, it was shown that chronic treatment by BZ partially restored the activity of COX and of succinate dehydrogenase in mTOR-deficient muscle, and upregulated gene expression of PGC-1α and of several mitochondrial genes including CPT1, MCAD, and LCAD, as well as COX and citrate synthase [133].