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Molecular Neuropathology of TDP-43 Proteinopathies
AbstractThe 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.
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Neumann, M. Molecular Neuropathology of TDP-43 Proteinopathies. Int. J. Mol. Sci. 2009, 10, 232-246.View more citation formats
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.