期刊
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
卷 10, 期 1, 页码 232-246出版社
MDPI
DOI: 10.3390/ijms10010232
关键词
TDP-43; frontotemporal dementia; amyotrophic lateral sclerosis; molecular neuropathology
资金
- Deutsche Forschungsgemeinschaft [SFB 596]
- German Federal Ministry of Education and Research [01GI0704]
- Stavros-Niarchos Foundation
- Synapsis Foundation
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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