Among the age-dependent protein aggregation disorders, nine neurodegenerative diseases
are caused by expansions of CAG repeats encoding polyglutamine (polyQ) tracts. We …

Protein misfolding and aggregation are a hallmark of several neurodegenerative diseases
(ND s). However, how protein aggregation leads to cytotoxicity and neurodegeneration is …

Abstract TDP-43 is a DNA/RNA binding protein associated with TDP-43 proteinopathies.
Many mutations have been identified in the flexible C-terminal region, which is implicated in …

Huntington disease (HD) is an inherited neurodegenerative disease caused by a CAG
expansion in the HTT gene. Using yeast two-hybrid methods, we identified a large set of …

During normal aging or neurodegenerative diseases, neuronal survival and function depend
on protein homeostasis, which is regulated by multiple mechanisms, including the …

Maintaining protein homeostasis (proteostasis) is essential for cellular health and is
governed by a network of quality control machinery comprising over 800 genes. When …

Alternative splicing (AS) appears to be altered in Huntington's disease (HD), but its
significance for early, pre-symptomatic disease stages has not been inspected. Here, taking …

PRPF40A plays an important role in the regulation of pre-mRNA splicing by mediating
protein-protein interactions in the early steps of spliceosome assembly. By binding to …

Ubiquitin-specific protease 19 (USP19) is one of the deubiquitinating enzymes (DUBs)
involved in regulating the ubiquitination status of substrate proteins. There are two major …

Huntington's disease (HD) is caused by aberrant expansion of polyglutamine (polyQ) in the
N-terminus of huntingtin (Htt). Our previous study has demonstrated that HSP90 is involved …