Mutations in fused in sarcoma (FUS) are a cause of familial amyotrophic lateral sclerosis (fALS).
Patients carrying point mutations in the C‐terminus of FUS show neuronal cytoplasmic …

Gene modifications in animal models have been greatly facilitated through the application
of targeted genome editing tools. The prokaryotic CRISPR/Cas9 type II genome editing …

Loss of function mutations in progranulin cause tau-negative frontotemporal lobar degeneration
with ubiquitin-positive inclusions. A major protein component of these inclusions is TDP-…

Mutations in the Tar DNA binding protein of 43 kDa (TDP-43; TARDBP) are associated with
amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 + …

Mitochondria provide ATP, maintain calcium homeostasis, and regulate apoptosis. Neurons,
due to their size and complex geometry, are particularly dependent on the proper …

Background The most frequent genetic cause of frontotemporal lobar degeneration (FTLD)
and amyotrophic lateral sclerosis (ALS) is the expansion of a GGGGCC hexanucleotide …

Methylene blue is an FDA approved compound with a variety of pharmacologic activities. It
inhibits aggregation of several amyloidogenic proteins known to be deposited in …

Alzheimer's disease is the most frequent dementia. Pathologically, Alzheimer's disease is
characterized by the accumulation of senile plaques composed of amyloid β‐peptide (Aβ). …

The CRISPR CRISPR / Cas Cas system identified in archaea archaea has been adopted
and optimized for genome genome editing purposes in zebrafish zebrafish . In vitro In vitro …

Aggregation of the Tar DNA-binding protein of 43 kDa (TDP-43) is a pathological hallmark
of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) and likely …