Scientists discover the molecular mechanisms associated with "frozen disease"

Scientists discover the molecular mechanisms associated with "frozen disease"

March 21, 2018 Source: Xinhuanet

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An international team reported on the 18th that they discovered a new molecular mechanism related to "frozen disease" that can help understand the pathogenesis of this disease and the development of related targeted drugs.

"Frozen disease" medical name is amyotrophic lateral sclerosis, a neurodegenerative disease that affects motor-related nerve cells in the brain and spinal cord, causing motor neurons to die, making the brain unable to control muscle movement. The main clinical manifestation is that the muscles gradually shrink and weak, and the patient will eventually die due to respiratory failure. The famous physicist who died recently, Hawking, was diagnosed with the disease at the age of 21 and eventually fought for 55 years.

Neuroscientists and Israeli counterparts at the University of Montreal in Canada reported on the Internet in the British journal Brain on the 18th that in an eight-year study, they focused on the removal of a protein called TDP-43 from nerve cells. Impact. Studies have found that deletion of the TDP-43 protein leads to a variant of another protein called hnRNPA1, which was not mentioned in the previous literature.

TDP-43 of normal nerve cells is present in the nucleus. Previous studies have shown that TDP-43 is a protein associated with the pathogenesis of amyotrophic lateral sclerosis. Deletion of TDP-43 function in the nucleus or abnormal TDP-43 protein aggregates in the cytoplasm may be a key factor in pathogenesis.

"Amyotrophic lateral sclerosis (pathogenesis) is very complex, and our results provide important information for future drug targets and the development of biomarkers for rapid disease detection and disease tracking," said the first author of the paper, University of Montreal. Adrian researcher Yard Emmanuel Desai said.

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