MTOR and Huntington disease: mTOR signaling is upregulated in the muscle of an HD mouse model [95] and in the cortex of mice overexpressing TAR DNA-binding protein 43 (TDP43) [102]; in contrast, it is downregulated in the muscle of a mouse model of valosin-containing protein-associated inclusion body myopathy [14], in the striatum of HD patients and mice [44], and in the spinal cord of transgenic mice expressing mutant SOD1 [109].