Molecular Neuropathology of TDP-43 Proteinopathies

作者：Neumann Manuela^*

来源：International Journal of Molecular Sciences, 2009, 10(1): 232-246.

DOI：10.3390/ijms10010232

• TDP-43
• frontotemporal dementia
• amyotrophic lateral sclerosis
• 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.

• 出版日期2009-1