TAR-DNA binding protein 43 in Pick disease
TAR-DNA binding protein 43 in Pick disease
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DOI：
10.1097/nen.0b013e3181609361
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发表时间：
2008-01-01
影响因子：
3.2
通讯作者：
Frosch, Matthew P.
中科院分区：
文献类型：
--
作者：
Freeman, Stefanie H.;Spires-Jones, Tara;Frosch, Matthew P.
Pick病是一种额颞部痴呆，其特征是额叶和颞叶萎缩，神经元丢失，神经胶质增生，α-13晶体蛋白和神经细丝阳性的神经元气球，以及tau和泛素阳性的Pick小体的存在。TDP-43(TDP-43)是其他神经退行性疾病泛素化包涵体的一种成分，包括额颞叶变性伴泛素化包涵体和肌萎缩侧索硬化症。用免疫组织化学方法检测15例PID，其中5例Pick小体和胞浆内小包涵体均为泛素、tau和TDP-43染色。PID中TDP-43包涵体的存在提示TDP-43积聚可能是许多神经退行性疾病的重要组成部分，仅在某些PID病例中存在TDP-43可能提示疾病发展的不同途径。
Pick disease (PiD) is a frontotemporal dementia characterized by frontal and temporal atrophy, neuronal loss, gliosis, ballooned neurons that are positive for alpha-13 crystallin and neurofilament, and the presence of tau- and ubiquitin-positive Pick bodies. TAR-DNA binding protein 43 (TDP-43) has been found to be a component of ubiquitinated inclusions in other neurodegenerative diseases, including frontotemporal lobar degeneration with ubiquitinated inclusions and amyotrophic lateral sclerosis. Fifteen cases of PiD were examined using imimmohistochemical methods, and 5 cases with both Pick bodies and smaller intracytoplasmic inclusions that showed staining for ubiquitin, tau, and TDP-43 were observed. The presence of TDP-43 inclusions in PiD suggests that TDP-43 accumulation may be an important component of many neurodegenerative diseases, and that its presence in only some cases of PiD may indicate different pathways of disease development.