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Int. J. Mol. Sci. 2009, 10(1), 232-246; doi:10.3390/ijms10010232
Review

Molecular Neuropathology of TDP-43 Proteinopathies

Received: 19 December 2008; in revised form: 6 January 2009 / Accepted: 8 January 2009 / Published: 9 January 2009
(This article belongs to the Special Issue Advances in Molecular Neuropathology)
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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; frontotemporal dementia; amyotrophic lateral sclerosis; molecular neuropathology TDP-43; frontotemporal dementia; amyotrophic lateral sclerosis; molecular neuropathology
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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MDPI and ACS Style

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

AMA Style

Neumann M. Molecular Neuropathology of TDP-43 Proteinopathies. International Journal of Molecular Sciences. 2009; 10(1):232-246.

Chicago/Turabian Style

Neumann, Manuela. 2009. "Molecular Neuropathology of TDP-43 Proteinopathies." Int. J. Mol. Sci. 10, no. 1: 232-246.


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