TAR-DNA binding protein 43 in Pick disease

被引:68
作者
Freeman, Stefanie H. [1 ,2 ,3 ,4 ,5 ]
Spires-Jones, Tara [2 ,3 ,4 ,5 ]
Hyman, Bradley T. [1 ,2 ,3 ,4 ,5 ]
Growdon, John H. [2 ,3 ,4 ,5 ]
Frosch, Matthew P. [1 ,2 ,3 ,4 ,5 ]
机构
[1] Massachusetts Gen Hosp, Dept Pathol, CS Kubik Lab Neuropathol, Boston, MA 02114 USA
[2] Massachusetts Gen Hosp, Alzheimers Res Unit, MassGen Inst Neurodegenerat Dis, Boston, MA 02114 USA
[3] Massachusetts Gen Hosp, Dept Neurol, Boston, MA 02114 USA
[4] Massachusetts Gen Hosp, Dept Neurol, Boston, MA 02114 USA
[5] Harvard Univ, Sch Med, Boston, MA USA
关键词
frontotemporal dementia; immunohistochemistry; neurodegeneration; Pick bodies; Pick disease; TDP-43;
D O I
10.1097/nen.0b013e3181609361
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:62 / 67
页数:6
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