In a new study, researchers from the Case Western Reserve University School of Medicine have identified the structure of protein fibrils linked to a hereditary form of human prion disease.
New Clues Uncovered about Toxic Protein Structures in Neurodegenerative Diseases
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March 15, 2021
Transactive response DNA-binding protein-43 (TDP-43) is a soluble protein that interacts with nucleic acids. However, in several neurodegenerative disorders, this protein forms large, harmful rope-like clumps. These abnormal structures, known as amyloid fibrils, are a signature of brain pathology in amyotrophic lateral sclerosis (ALS). Similar inclusions have also been found in several other disorders, including Alzheimer’s disease, cerebral age-related TDP-43 with sclerosis, dementia with Lewy bodies, hippocampal sclerosis, Huntington’s disease, and chronic traumatic encephalopathy.
By using cryo-electron microscopy, scientists at the Case Western Reserve University School of Medicine were able to determine the structures of TDP-43. This structural insight provides clues as to how these toxic proteins clump and spread between nerve cells in the b
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CLEVELAND Scientists at the Case Western Reserve University School of Medicine have determined the structure of protein fibrils linked to Lou Gehrig s disease and other neurodegenerative disorders findings that provide clues to how toxic proteins clump and spread between nerve cells in the brain.
Their results may also lead to developing drugs to treat diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). These devastating brain disorders that affect tens of thousands of Americans?are on the rise worldwide, and there are no effective treatments to stop their progression, said Witold Surewicz, a professor in the Department of Physiology and Biophysics at the School of Medicine and the study s senior author.
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