EBSCO Logo
Connecting you to content on EBSCOhost
Results
Title

TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander.

Authors

Banks, Gareth; Kuta, Anna; Isaacs, Adrian; Fisher, Elizabeth

Abstract

In 2006 the protein TDP-43 was identified as the major ubiquitinated component deposited in the inclusion bodies found in two human neurodegenerative diseases, amyotrophic lateral sclerosis and frontotemporal lobar degeneration. The pathogenesis of both disorders is unclear, although they are related by having some overlap of symptoms and now by the shared histopathology of TDP-43 deposition. Now, in 2008, several papers have been published in quick succession describing mutations in the TDP-43 gene, showing they can be a primary cause of amyotrophic lateral sclerosis. There are many precedents in neurodegenerative disease in which rare single-gene mutations have given great insight into understanding disease processes, which is why the TDP-43 mutations are potentially very important.

Subjects

NEURODEGENERATION; AMYOTROPHIC lateral sclerosis; NEUROMUSCULAR diseases; MOTOR neuron diseases; HUMAN heredity

Publication

Mammalian Genome, 2008, Vol 19, Issue 5, p299

ISSN

0938-8990

Publication type

Academic Journal

DOI

10.1007/s00335-008-9117-x

EBSCO Connect | Privacy policy | Terms of use | Copyright | Manage my cookies
Journals | Subjects | Sitemap
© 2025 EBSCO Industries, Inc. All rights reserved