We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies.
- Authors
Geser, Felix; Lee, Virginia M.-Y.; Trojanowski, John Q.
- Abstract
It is now established that pathological transactive response DNA-binding protein with a Mr of 43 kD (TDP-43) on sodium dodecyl sulfate-polyacrylamide gel electrophoresis is the major disease protein in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) with ubiquitin-positive inclusions (now known as FTLD-TDP). In fact, the discovery of pathological TDP-43 solidified the idea that these disorders are multi-system diseases and this led to the concept of a TDP-43 proteinopathy as a spectrum of disorders comprised of different clinical and pathological entities extending from ALS to ALS with cognitive impairment/dementia and FTLD-TDP without or with motor neuron disease (FTLD-MND). These align along a broad disease continuum sharing similar pathogenetic mechanisms linked to pathological TDP-43. We here review salient findings in the development of a concept of TDP-43 proteinopathy as a novel group of neurodegenerative diseases similar in concept to α-synucleinopathies and tauopathies.
- Subjects
AMYOTROPHIC lateral sclerosis; POLYACRYLAMIDE gel electrophoresis; DEMENTIA; NEURODEGENERATION; MOTOR neuron diseases; GEL electrophoresis
- Publication
Neuropathology, 2010, Vol 30, Issue 2, p103
- ISSN
0919-6544
- Publication type
Article
- DOI
10.1111/j.1440-1789.2009.01091.x