Penn Researchers Make Major Advancement in Lou Gehrig’s Disease and FTD

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Omschrijving
Researchers at the University of Pennsylvania School of Medicine have discovered the major disease protein for two neurodegenerative disorders: a type of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), also called Lou Gehrig’s disease. A protein called TDP-43 was found to accumulate abnormally in post-mortem brain tissue from individuals diagnosed with either disease. The misfolded, disease protein was recovered from only affected central nervous system regions, which include the hippocampus, neocortex, and spinal cord.

These findings open up new avenues of research into how the crumpling, or misfolding, of specific brain proteins (TDP-43) leads to strange, and sometimes even criminal behavior (in FTD), as well as paralysis (in ALS), which depends upon whether these toxic waste products get dumped in the frontal and temporal lobes (the part of brain controlling judgment and comportment), or the spinal cord motor neurons (the control centers regulating the ability to walk, run, and other types of movement).

 

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