CHMP2B and juvenile Huntington disease: For example, previous studies have shown that in sporadic AD there is a profound reduction in the levels of Beclin-1 [34], while the neurodegenerative process in familial forms of fronto-temporal dementia and ALS has been linked to mutations in charged multivesicular body protein-2B (CHMP2B) [86], [87], [88], and in Huntington's Disease polyglutamate aggregates trap mTor and disrupt autophagy [80].