SQSTM1 and juvenile Huntington disease: In amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), mutant TBK1 reduces SQSTM1/p62 phosphorylation and compromises cargo binding and clearance, responsible for related neurotoxicity.157 It has found that ULK1 can regulate ATP14 phosphorylation, which displays a special role in the context of Huntington’s disease.