期刊
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
卷 68, 期 11, 页码 1167-1176出版社
OXFORD UNIV PRESS INC
DOI: 10.1097/NEN.0b013e3181baacec
关键词
alpha B-Crystallin; Astrocyte; Capillary basal lamina; Frontotemporal lobar degeneration; Immunoelectron microscopy; Lewy body disease; TDP-43
资金
- NIA NIH HHS [P50 AG25711, P01 AG03949, P50 AG016574-109002, P01 AG003949-250009, P01 AG017216-109001, P01 AG017216, P50 AG025711, P50 AG16574, P50 AG016574, P50 AG025711-059003, P01 AG17216, P01 AG003949] Funding Source: Medline
- NINDS NIH HHS [P50 NS40256, P50 NS040256, P50 NS040256-10] Funding Source: Medline
We describe novel transactivation response DNA-binding protein of 43 kd (TDP-43)-positive structures in the brains of patients with frontotemporal lobar degeneration with ubiquitin-positive inclusions and familial Lewy body disease. The TDP-43 immunohistochemistry revealed small round structures closely associated with small blood vessels. By immunoelectron microscopy, these TDP-43-positive structures were unmyelinated cell processes located adjacent to and sometimes enclosed by the capillary basal lamina. Some processes protruded from outside of the vascular basal lamina to a position beneath the basal lamina. The processes contained 10- to 17-nm-diameter straight filaments or filaments coated with granular material similar to those described in neurites in frontotemporal lobar degeneration with ubiquitin-positive inclusions and other disorders. In some of the abnormal structures, electron-dense material formed paracrystalline arrays composed of TDP-43. The inclusions were variably positive by immunostaining for the small heat shock protein alpha B-crystallin and less often glial fibrillary acidic protein. Bundles of astrocytic glial fibrils characteristic of reactive astrocytes were often found in proximity, but glial fibrils were negative for TDP-43. These data suggest that these processes are astrocytic end-feet with abnormal TDP-43 fibrillary inclusions. The significance of this novel TDP-43 microvasculopathy on blood-brain barrier integrity wan-ants further investigation.
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