Ubiquitin acts as a versatile cellular signal that controls a wide range of biological processes
including protein degradation, DNA repair, endocytosis, autophagy, transcription, immunity …

Deubiquitinases (DUBs) have fundamental roles in the ubiquitin system through their ability
to specifically deconjugate ubiquitin from targeted proteins. The human genome encodes at …

Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is
the most common inherited spinocerebellar ataxia and one of many polyglutamine …

The aggresome pathway is activated when proteasomal clearance of misfolded proteins is
hindered. Misfolded polyubiquitinated protein aggregates are recruited and transported to …

p97/VCP/Cdc48 is one of the best-characterized type II AAA (ATPases associated with
diverse cellular activities) ATPases. p97 is suggested to be a ubiquitin-selective chaperone …

Ataxia is a neurological disorder characterized by loss of control of body movements.
Spinocerebellar ataxia (SCA), previously known as autosomal dominant cerebellar ataxia, is …

Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA3), may be the most common dominantly inherited ataxia in the world. Here I will review …

Machado–Joseph disease is the most frequently found dominantly-inherited cerebellar
ataxia. Over-repetition of a CAG trinucleotide in the MJD1 gene translates into a …

Polyubiquitination of misfolded proteins, especially K63-linked polyubiquitination, is thought
to be associated with the formation of inclusion bodies. However, it is not well explored …

To maintain protein homeostasis in the ER, an ER protein quality control system retains
unfolded polypeptides and misassembled membrane proteins, allowing only properly folded …