自噬
陶氏病
细胞生物学
生物
蛋白质稳态
溶酶体
内吞循环
生物发生
HEK 293细胞
内体
ATG5型
好斗的
串扰
死孢子体1
泛素
程序性细胞死亡
袋3
蛋白酶体
TFEB
突变
神经退行性变
基因沉默
RNA干扰
功能(生物学)
内吞作用
磷酸化
粒体自噬
应力颗粒
细胞培养
巴非霉素
细胞
mTORC1型
蛋白质降解
作者
Celeste M. Karch,Farzaneh S. Mirfakhar
出处
期刊:Autophagy
[Taylor & Francis]
日期:2026-05-06
卷期号:: 1-3
标识
DOI:10.1080/15548627.2026.2669685
摘要
Tauopathies are characterized by the accumulation of misfolded tau and lysosomal dysfunction, yet whether defects in the autophagy-lysosome pathway are causal or secondary remains unclear. Recent work using human iPSC-derived neurons harboring the MAPT p.R406W mutation demonstrates that pathogenic tau is sufficient to disrupt lysosomal function upstream of tau accumulation. Tau species are differentially processed within lysosomes, with phosphorylated tau retained at the lysosomal membrane, consistent with a barrier to efficient cargo processing. Importantly, pharmacologic activation of autophagy restores degradative capacity and reduces tau burden without rescuing lysosomal motility, suggesting that trafficking and degradation represent separable axes of lysosomal biology. These findings position tau as an active disruptor of proteostasis and define a degradative bottleneck that shares features with lysosomal storage disorders. Together, this work reframes autophagy dysfunction in tauopathy as a modular defect with distinct therapeutic entry points.
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