期刊
CURRENT OPINION IN NEUROBIOLOGY
卷 17, 期 5, 页码 548-555出版社
CURRENT BIOLOGY LTD
DOI: 10.1016/j.conb.2007.08.005
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资金
- NIA NIH HHS [AG10124, P01 AG017586, T32 AG000255, AG09215, P01 AG009215, P30 AG010124, AG17586, T32 AG000255-02] Funding Source: Medline
Over the past decade, it has become clear that there is a significant overlap in the clinical spectrum of frontotemporal lobar degeneration and amyotrophic lateral sclerosis (ALS). The identification of TDP-43 as the major disease protein in the pathology of both frontotemporal lobar degeneration with ubiquitin inclusions and ALS provides the first molecular link for these diseases. Pathological TDP-43 is abnormally phosphorylated, ubiquitinated, and cleaved to generate carboxy-terminal fragments in affected brain regions. The normal nuclear expression of TDP-43 is also reduced leading to the hypothesis that sequestration of TDP-43 in pathological inclusions contributes to disease pathogenesis. Thus, TDP-43 is the newest member of the growing list of neurodegenerative proteinopathies, but unique in that it lacks features of brain amyloidosis.
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