期刊
ACTA NEUROPATHOLOGICA
卷 116, 期 2, 页码 205-213出版社
SPRINGER
DOI: 10.1007/s00401-008-0408-9
关键词
Alzheimer's disease; amyotrophic lateral sclerosis; frontotemporal lobar degeneration with ubiquitinated inclusions; immunoelectron microscopy; Lewy body disease; Pick's disease; TAR DNA-binding protein of 43 kDa (TDP-43)
资金
- NIA NIH HHS [P50 AG025711-059003, P01-AG03949, P01 AG003949, P50-AG25711, P50 AG016574, P01 AG003949-250009, P50-AG16574, P01-AG17216, P50 AG025711, P01 AG017216, P01 AG017216-09, P50 AG016574-109002] Funding Source: Medline
- NINDS NIH HHS [P50 NS040256-10, P50-NS40256, P50 NS040256] Funding Source: Medline
Using post-embedding immunogold electron microscopy, TAR DNA-binding protein of 43 kDa (TDP-43) was localized to neuronal cytoplasmic (NCI) and intranuclear (NII) inclusions, as well as unmyelinated neurites, in frontotemporal lobar degeneration with ubiquitinated inclusions (FTLD-U), amyotrophic lateral sclerosis (ALS), Alzheimer's (AD), Pick's disease (PiD) and Lewy body disease (LBD). The TDP-43 immunoreactive structures were morphologically heterogeneous. The most common was characterized by bundles of 10-20 nm diameter straight filaments with electron dense granular material within NCI, NII and neurites. This type of pathology was found in FTLD-U, ALS and some cases of AD. Less often, inclusions in neuritic processes of FTLD-U and some cases of AD contained 10-17 nm diameter straight filaments without granular material. A final type of TDP-43 immunoreactivity was labeling of filaments and granular material associated with tau filaments in neurofibrillary tangles of AD and Pick bodies of PiD or alpha-synuclein filaments in Lewy bodies of LBD. The results suggest that TDP-43 is the primary component of the granulofilamentous inclusions in FTLD-U and ALS. Similar inclusions sometimes accompany filamentous aggregates composed of other abnormal proteins in AD, PiD and LBD.
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