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.
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CITATION STYLE
Neumann, M. (2009, January). Molecular neuropathology of TDP-43 proteinopathies. International Journal of Molecular Sciences. https://doi.org/10.3390/ijms10010232