Neurodegenerative Disease Tauopathies

被引:11
|
作者
Creekmore, Benjamin C. [1 ]
Watanabe, Ryohei [1 ]
Lee, Edward B. [1 ]
机构
[1] Univ Penn, Translat Neuropathol Res Lab, Dept Pathol & Lab Med, Perelman Sch Med, Philadelphia, PA 19104 USA
关键词
Alzheimer's disease; AD; frontotemporal lobar degeneration; FTLD; microtubule-associated protein tau; MAPT; tauopathy; proteostasis; cryogenic electron microscopy; cryo-EM; PAIRED HELICAL FILAMENTS; ALZHEIMERS-DISEASE; PROTEIN-TAU; PATHOLOGICAL TAU; NEUROFIBRILLARY TANGLES; BETA-AMYLOIDOSIS; PHOSPHORYLATION; AUTOPHAGY; BRAINS; TRANSMISSION;
D O I
10.1146/annurev-pathmechdis-051222-120750
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
Tauopathies are a diverse group of progressive and fatal neurodegenerative diseases characterized by aberrant tau inclusions in the central nervous system. Tau protein forms pathologic fibrillar aggregates that are typically closely associated with neuronal cell death, leading to varied clinical phenotypes including dementia, movement disorders, and motor neuron disease. In this review, we describe the clinicopathologic features of tauopathies and highlight recent advances in understanding the mechanisms that lead to spread of pathologic aggregates through interconnected neuronal pathways. The cell-to-cell propagation of tauopathy is then linked to posttranslational modifications, tau fibril structural variants, and the breakdown of cellular protein quality control.
引用
收藏
页码:345 / 370
页数:26
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