Phase to Phase with TDP-43
作者： Yulong Sun , Avijit Chakrabartty
DOI: 10.1021/ACS.BIOCHEM.6B01088
关键词:
摘要: TDP-43 is a dimeric nuclear protein that plays central role in RNA metabolism. In recent years, this has become focal point of research the amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD) disease spectrum, as pathognomonic inclusions within affected neurons contain post-translationally modified TDP-43. A key question involves determining mechanisms triggers cause to form pathological aggregates. This review gives brief overview physiological roles focuses on structural features its domains how they may contribute normal function disease. special emphasis placed C-terminal prion-like region thought be implicated pathology, it where nearly all ALS/FTD-associated mutations reside. Recent studies domain revealed crucial formation phase-separated liquid droplets through partially populated α-helix. ...
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