A pathogenic Tau mutation drives autophagy-lysosome dysfunction that limits Tau degradation in a model of frontotemporal dementia

Mar 31, 2026Nature communications

A harmful Tau mutation may cause problems in cell waste removal that reduce Tau breakdown in frontotemporal dementia

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Abstract

A disease-causing mutation in the MAPT gene disrupts multiple steps of the -lysosome pathway in human neurons.

  • Tau accumulates in neurodegenerative diseases called , raising questions about the role of cellular clearance mechanisms.
  • Mutant Tau neurons show increased levels of both Tau and phosphorylated Tau within dysfunctional lysosomes.
  • Defects in lysosomal function include reduced motility and impaired fusion with autophagosomes, leading to the accumulation of undegraded cellular materials.
  • Enhancing autophagy pharmacologically improves the clearance of cellular debris and lowers Tau levels, although it does not restore lysosomal motility.
  • These findings indicate that mutant Tau affects cellular clearance processes directly, and boosting autophagy could help manage Tau levels in tauopathies.

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Key numbers

67.1%
Increase in lysosomes free of Tau
Percentage of lysosomes free of Tau in G2-567 treated p.R406W neurons.
37
Decrease in total Tau levels
Mean intensity of total Tau per cell in G2-567 treated p.R406W neurons.
56
Decrease in pTau levels
Mean intensity of pTau per cell in G2-567 treated p.R406W neurons.

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