Stas (Stanislav) Engel

Amyotrophic lateral sclerosis (ALS) – is a neurodegenerative disease characterized by a gradual degeneration and death of motor neurons (MNs). A prominent ALS pathogenic protein is a ubiquitous enzyme superoxide dismutase 1 (SOD1), which is responsible for a significant fraction of familial and probably some sporadic cases. In ALS, SOD1 undergoes a noxious structural transformation, so-called misfolding, as a result of mutation or environmental stress, such as oxidative stress, rendering SOD1 neurotoxic. We have identified a pathogenic epitope exposed in misfolded SOD1, which appears to contribute to SOD1 neurotoxicity in ALS. Our strategy is to target this pathogenic SOD1 epitope, using a wide variety of tools, including immunization, gene therapy, effector cell-retargeting therapy and small-molecule compounds, to develop novel mechanism-based treatment/prophylactic strategies for ALS. We also extend the application of the novel therapeutic approached to other common neurodegenerative disease, such as Alzheimer's and Parkinson's diseases.

Email: engels@bgu.ac.il

Personal website: https://engels45.wixsite.com/engelslab