721 results found
Modeling 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…
Project Ongoing
See the project and researchersDNA damage driven motor disturbance in ALS: An ERVK integrase transgenic mouse model
Project Overview
Endogenous retrovirus-K (ERVK) is a genomic viral symbiont that contributes towards ALS neuropathology. Our collaboration proposes to examine how ERVK integrase (IN) enzyme activity drives DNA damage, TDP-43 deregulation and neuronal loss, leading to motor disturbances using a novel ERVK IN transgenic mouse model system. The intent of this proposal…
Project Ongoing
See the project and researchersRemote ischemic preconditioning treatment in amyotrophic lateral sclerosis
Project Overview
RATIONALE: There are no effective disease modifying therapies for amyotrophic lateral sclerosis (ALS). Complete understanding of the physiopathology of ALS is lacking, and single-target therapies have failed clinical translation. The overarching goal of this grant application is to test the hypothesis that remote ischemic preconditioning (REIP), a non-invasive, endogenous, pleiotropic…
Project Ongoing
See the project and researchersDeepening our understanding of G3BP1 and its paralog G3BP2 to facilitate accurate therapeutic development for ALS/FTD
Project Overview
While significant efforts have been made worldwide, the development of an effective therapy to significantly slow or halt ALS disease progression has remained elusive. This is due in part to our incomplete understanding of the molecular mechanisms and aberrant processes that lead to disease. TDP-43 (TAR DNA binding protein 43)…
Project Ongoing
See the project and researchersThe impact of ALS disease variants on regulation of heat shock protein life cycles – Implications for neuronal proteostasis
Project Overview
The abnormal accumulation of misfolded proteins and aggregates is the hallmark of multiple forms of ALS and other neurodegenerative diseases. The toxicity of these aberrant proteins is compounded by deficiency in mechanisms regulating protein quality control and alterations they cause in the metabolism of mRNAs that sustain neuronal functionality. A…