The endoplasmic reticulum (ER) is an important organelle involved in cellular homeostasis
and control of protein quality. Unfolded protein response (UPR) is a cellular response to ER …

At present, there are very few therapeutic options for patients affected by amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD). However, almost all patients affected by …

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease
characterised by the degeneration of upper and lower motor neurons. Recent advances in …

Since the discovery that 43 kDa TAR DNA binding protein (TDP-43) is involved in
neurodegeneration, studies of this protein have focused on the global effects of TDP-43 …

Introduction Neurofibrillary tangles (NFT) composed of Tau are hallmarks of
neurodegeneration in Alzheimer disease. Transgenic mice expressing full-length pro …

C. elegans and D. rerio expressing mutant TAR DNA Binding Protein 43 (TDP-43) are
powerful in vivo animal models for the genetics and pharmacology of amyotrophic lateral …

Impaired proteostasis is one of the main features of all amyloid diseases, which are
associated with the formation of insoluble aggregates from amyloidogenic proteins. The …

The DNA/RNA-binding proteins TDP-43 and FUS are found in protein aggregates in a
growing number of neurodegenerative diseases, including amyotrophic lateral sclerosis …

Brain has exceptional high requirement for energy metabolism with glucose as the exclusive
energy source. Decrease of brain energy metabolism and glucose uptake has been found in …

Following the discovery of the involvement of the ribonucleoprotein TDP‐43 in amyotrophic
lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), a major research …