TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …

The endoplasmic reticulum (ER) is an important organelle involved in protein quality control
and cellular homeostasis. The accumulation of unfolded proteins leads to an ER stress …

Protein homeostasis (proteostasis) requires the timely degradation of misfolded proteins and
their aggregates by protein quality control (PQC), of which molecular chaperones are an …

Mitochondria and the endoplasmic reticulum (ER) form tight structural associations and
these facilitate a number of cellular functions. However, the mechanisms by which regions of …

The unfolded protein response (UPR) is a homeostatic mechanism by which cells regulate
levels of misfolded proteins in the endoplasmic reticulum (ER). Although it is well …

Stress granule (SG) assembly is a conserved cellular strategy to minimize stress-related
damage and promote cell survival. Beyond their fundamental role in the stress response …

The occurrence of repeat-associated non-ATG (RAN) translation, an atypical form of
translation of expanded repeats that results in the synthesis of homopolymeric expansion …

The normally soluble TAR DNA-binding protein 43 (TDP-43) is found aggregated both in
reversible stress granules and in irreversible pathogenic amyloid. In TDP-43, the low …

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …

The discovery that prion protein can misfold into a pathological conformation that encodes
structural information capable of both propagation and inducing severe neuropathology has …