Amyotrophic lateral sclerosis (ALS) is manifested as skeletal muscle denervation, loss of
motor neurons and finally severe respiratory failure. Mutations of RNA-binding protein FUS …

Mutations in RNA-binding proteins, including fused in sarcoma (FUS) and TAR DNA-binding
protein 43 (TDP-43, encoded by TARDBP), are associated with sporadic and familial …

Although physiological differences among neuromuscular junctions (NMJs) have long been
known, NMJs have usually been considered as one type of synapse, restricting their …

Studies examining the etiology of motoneuron diseases usually focus on motoneuron death
as the defining pathophysiology of the disease. However, impaired neuromuscular …

Studies of neuromuscular synaptic structure and function have, historically, given general
insights into synaptic mechanisms, whose principles were then extended to most other …

Neuromuscular junction (NMJ) disruption is an early pathogenic event in amyotrophic lateral
sclerosis (ALS). Yet, direct links between NMJ pathways and ALS-associated genes such as …

Mutations in the RNA-binding protein FUS (fused in sarcoma) are linked to amyotrophic
lateral sclerosis (ALS), but the mechanism by which these mutants cause motor neuron …

Mutations in FUS are causative for amyotrophic lateral sclerosis with a dominant mode of
inheritance. In trying to model FUS-amyotrophic lateral sclerosis (ALS) in mouse it is clear …

FUS (fused in sarcoma) mislocalization and cytoplasmic aggregation are hallmark
pathologies in FUS-related amyotrophic lateral sclerosis and frontotemporal dementia. Many …

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that leads to the
eventual death of motor neurons. Described cases of familial ALS have emphasized the …