Format

Send to

Choose Destination
Int J Mol Sci. 2009 Jan;10(1):232-46. doi: 10.3390/ijms10010232. Epub 2009 Jan 9.

Molecular neuropathology of TDP-43 proteinopathies.

Author information

1
Institute of Neuropathology, University Hospital of Zurich, Switzerland. Manuela.neumann@usz.ch

Abstract

The identification of TDP-43 as the major component of the pathologic inclusions in most forms of sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS) resolved a long-standing enigma concerning the nature of the ubiquitinated disease protein under these conditions. Anti-TDP-43 immunohistochemistry and the recent development of novel tools, such as phosphorylation-specific TDP-43 antibodies, have increased our knowledge about the spectrum of pathological changes associated with FTLD-U and ALS and moreover, facilitated the neuropathological routine diagnosis of these conditions. This review summarizes the recent advances in our understanding on the molecular neuropathology and pathobiology of TDP-43 in FTLD and ALS.

KEYWORDS:

TDP-43; amyotrophic lateral sclerosis; frontotemporal dementia; molecular neuropathology

PMID:
19333444
PMCID:
PMC2662455
DOI:
10.3390/ijms10010232
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for PubMed Central
Loading ...
Support Center