Login to MyKarger

New to MyKarger? Click here to sign up.

Login with Facebook

Forgot Password? Reset your password

Authors, Editors, Reviewers

For Manuscript Submission, Check or Review Login please go to Submission Websites List.

Submission Websites List

Institutional Login (Shibboleth)

For the academic login, please select your country in the dropdown list. You will be redirected to verify your credentials.

Table of Contents
Vol. 16, No. 1, 2008
Issue release date: December 2007
Section title: Paper
Neurosignals 2008;16:41–51

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

Do you have an account?

Login Information

Contact Information

I have read the Karger Terms and Conditions and agree.


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

Copyright / Drug Dosage / Disclaimer

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.