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TDP-43 Proteinopathies: Neurodegenerative Protein Misfolding Diseases without Amyloidosis

Kwong L.K.a · Uryu K.a · Trojanowski J.Q.a, b · Lee V.M.-Y.a, b

Author affiliations

aCenter for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, and bInstitute on Aging, University of Pennsylvania School of Medicine, Philadelphia, Pa., USA

Corresponding Author

Virginia M.-Y. Lee, PhD, Center for Neurodegenerative Disease Research

Department of Pathology and Laboratory Medicine

University of Pennsylvania School of Medicine, HUP, Maloney 3rd Floor

36th and Spruce Streets, Philadelphia, PA 19104-4283 (USA)

Tel. +1 215 662 6399, Fax +1 215 349 5909, E-Mail vmylee@mail.med.upenn.edu

Related Articles for ""

Neurosignals 2008;16:41–51

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In this review, we summarize recent advances in understanding frontotemporal lobar degeneration (FTLD), amyotrophic lateral sclerosis (ALS) and related neurodegenerative disorders that are collectively known as TDP-43 proteinopathies, since transactive response DNA-binding protein 43 (TDP-43) was recently shown to be the major component of the ubiquitinated inclusions that are their pathological hallmarks. TDP-43 proteinopathies are distinct from most other neurodegenerative disorders because TDP-43 inclusions are not amyloid deposits. Besides TDP-43-positive inclusions, both sporadic and familial forms of FTLD and ALS have the pathologic TDP-43 signature of abnormal hyperphosphorylation, ubiquitination and C-terminal fragments in affected brain and spinal cord, suggesting that they share a common mechanism of pathogenesis. Thus, these findings support the concept that FTLD and ALS represent a clinicopathologic spectrum of one disease, that is, TDP-43 proteinopathy.

© 2008 S. Karger AG, Basel

Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: December 05, 2007
Issue release date: December 2007

Number of Print Pages: 11
Number of Figures: 2
Number of Tables: 0

ISSN: 1424-862X (Print)
eISSN: 1424-8638 (Online)

For additional information: http://www.karger.com/NSG

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