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Molecular Neuropathology of TDP-43 Proteinopathies

Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstr. 12, 8091 Zurich, Switzerland
Int. J. Mol. Sci. 2009, 10(1), 232-246; https://doi.org/10.3390/ijms10010232
Received: 19 December 2008 / Revised: 6 January 2009 / Accepted: 8 January 2009 / Published: 9 January 2009
(This article belongs to the Special Issue Advances in Molecular Neuropathology)
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. View Full-Text
Keywords: TDP-43; frontotemporal dementia; amyotrophic lateral sclerosis; molecular neuropathology TDP-43; frontotemporal dementia; amyotrophic lateral sclerosis; molecular neuropathology
MDPI and ACS Style

Neumann, M. Molecular Neuropathology of TDP-43 Proteinopathies. Int. J. Mol. Sci. 2009, 10, 232-246.

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