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Molecular Neuropathology of TDP-43 Proteinopathies
Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstr. 12, 8091 Zurich, Switzerland
Received: 19 December 2008; in revised form: 6 January 2009 / Accepted: 8 January 2009 / Published: 9 January 2009
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
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MDPI and ACS Style
Neumann, M. Molecular Neuropathology of TDP-43 Proteinopathies. Int. J. Mol. Sci. 2009, 10, 232-246.
Neumann M. Molecular Neuropathology of TDP-43 Proteinopathies. International Journal of Molecular Sciences. 2009; 10(1):232-246.
Neumann, Manuela. 2009. "Molecular Neuropathology of TDP-43 Proteinopathies." Int. J. Mol. Sci. 10, no. 1: 232-246.