Reviewing What is Known of TDP-43 Aggregation in Neurodegeneration

TDP-43 is one of the more recently discovered protein aggregates involved in neurodegenerative conditions. A few proteins in the body are capable of misfolding or otherwise becoming altered in ways that encourage other molecules of the same protein to do the same. Toxicity results, and it can spread as these altered proteins move from cell to cell. The condition most clearly associated with TDP-43 pathology is amyotrophic lateral sclerosis (ALS), but it appears to be involved in other forms of neurodegenerative disease as well. Researchers have made inroads into understanding how these aggregates form and disrupt normal cellular operations, but as yet little progress has been made towards development of a viable approach to therapy. TAR DNA binding protein 43 kDa (TDP-43) plays an important role in several essential cell functions. However, TDP-43 dysfunction has been implicated in the development of various brain diseases including amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration (FTLD), and limbic predominant age-related TDP-43 encephalopathy (LATE). Recent investigations into the individual components of TDP-43 pathology show how broader TDP-43 dysfunction may precede these disease end states, and therefore could help to explain why TDP-43 dysfunction continues to be implicated in a rapidly expanding category of neurodegenerative diseases. TDP-43 pathology is usually characterized by insoluble, hyperphosphorylated and ubiquinated ag...
Source: Fight Aging! - Category: Research Authors: Tags: Daily News Source Type: blogs