Abstract

TAR DNA binding protein-43 (TDP-43) is the main component of cytoplasmic aggregates in motor neurons of Amyotrophic Lateral Sclerosis (ALS) patients. TDP-43 aggregates have also been found in muscle cells of patients with Facioscapulohumeral dystrophy (FSHD), a disease caused by the aberrant expression of DUX4 protein. Interestingly, the expression of DUX4 has been demonstrated to induce TDP-43 aggregation in human FSHD myoblasts and healthy cells transfected with DUX4. Based on these premises, we decided to verify if DUX4 is expressed in cells of ALS patients and, if expressed, its possible involvement in TDP-43 aggregation.

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call

Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.