HSJ1 was proposed to directly participate in the degradation of CFTR as well as neurodegenerative disease related proteins, such as the amyotrophic lateral sclerosis (ALS) linked mutant protein superoxide dismutase 1 (SOD1) [86] and the expanded polyQ forms of spinocerebellar ataxia type 3-linked protein [87]. This evidence concerns the gene CFTR and amyotrophic lateral sclerosis.