Cited by 195Amyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatmentsAmyloidosis: Diagnosis, Treatment, Outcomes

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Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

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Geser, Felix, Lee, Virginia M.-Y., Trojanowski, John Q.·2010·Neuropathology

DOI 10.1111/j.1440-1789.2009.01091.xPMC PMC3131978

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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 alpha-synucleinopathies and tauopathies.

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Authors: Geser, F., Lee, V. M.-Y., & Trojanowski, J. Q.
Affiliations: University of Pennsylvania (US)
Corresponding author: Trojanowski, John Q.
ORCID: Geser, Felix
Lee, Virginia M.-Y.
Trojanowski, John Q.
Venue: Neuropathology
Publisher: Wiley
Published: 2010-04
Volume: 30
Issue: 2
Pages: 103-112
ISSN: 0919-6544 (ISSN-L)
1440-1789
DOI: 10.1111/j.1440-1789.2009.01091.x
PMC: PMC3131978
OpenAlex: W2125931517
Cited by: 195
References: 108
Citations by year: 2025: 4 · 2024: 11 · 2023: 15 · 2022: 11 · 2021: 8 · 2020: 8 · 2019: 12 · 2018: 8 · 2017: 10 · 2016: 10 · 2015: 12 · 2014: 10 · 2013: 16 · 2012: 24
FWCI: 3.01
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: doi.wiley.com/10.1002/tdm_license_1.1 (tdm, 1979 d)
Locations: Neuropathology (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
Funders: National Institute on Aging (P30AG010124)
Topics: Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Health Sciences › Medicine › Internal Medicine › Amyloidosis: Diagnosis, Treatment, Outcomes
Keywords: amyotrophic lateral sclerosis, frontotemporal lobar degeneration, TDP-43, TDP-43 proteinopathy, FTLD-TDP, disease continuum, neurodegenerative diseases, synucleinopathies, tauopathy, ubiquitin-positive inclusions
MeSH terms: Amyotrophic Lateral Sclerosis, Brain, DNA-Binding Proteins, Humans, Neurons, Frontotemporal Lobar Degeneration

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Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

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