770 results found
Rational targeting of FUS and TDP-43 protein assemblies for diagnostics and treatment of ALS
Project Overview
The project addresses a grand challenge in ALS, the integration of a multi-targeted and personalized approach to patients with a rational strategy focusing on a unifying pivotal mechanism of the disease. We seek to test the extended “prion hypothesis”, which suggests that ALS progression is driven by a self-propagating buildup…
Project Ongoing
See the project and researchersElucidating the basis for ubiquitination of misfolded ALS proteins by E3 ligase Enzymes
Project Overview
A pathologic feature in both sporadic and familial ALS patients is the presence of large neuronal inclusions enriched with misfolded proteins, including SOD1, TDP43 and FUS that are frequently modified with the small protein ubiquitin (Ub). This observation, also common to Parkinson’s disease, indicates impairment in the ubiquitin-proteasome system that…
Project Ongoing
See the project and researchersNeuromuscular proteins as potential biomarkers in ALS
Project Overview
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the loss of upper and lower motor neurons (MNs). Symptoms include muscle paralysis which rapidly progresses and ultimately results in respiratory failure within 2-5 years after diagnosis. Even if several genes causing ALS have been identified, still up to ~90%…
Project Ongoing
See the project and researchersAxonal degeneration as a therapeutic target for ALS
Project Overview
One of the neuron’s most impressive morphological features is their long axonal projections, which are the basis of the complex, precise, and fast-firing neuronal network. To protect these fragile structures, neurons possess mechanisms to actively regulate axonal survival and degeneration, that are independent of the programmed cell death of the…
Project Ongoing
See the project and researchersModeling ALS progression by applying neuroinformatics to a novel in vitro human 3D tri-culture model of the disease
Project Overview
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease in which the motor neurons degenerate, leading to symptoms of muscle weakness and progressive paralysis, resulting in the death of patients from respiratory failure. The development of disease modifying therapies have been largely unsuccessful due to the poor understanding into the mechanisms…