Molecular Neuropathology of TDP-43 Proteinopathies

• Main Author: Manuela Neumann
• Format: Article
• Language: English
• Published: MDPI AG 2009-01-01
• Series: International Journal of Molecular Sciences
• Subjects: TDP-43; frontotemporal dementia; amyotrophic lateral sclerosis; molecular neuropathology
• Online Access: http://www.mdpi.com/1422-0067/10/1/232/

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.