Author

Angelo Poletti

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Review Sep 2026

Chaperone-assisted selective autophagy (CASA) in polyglutamine-related diseases: From mechanisms to therapeutic perspectives.

Chaperone-assisted selective autophagy (CASA) is a crucial process aimed at maintaining proteostasis in several neurodegenerative diseases associated with protein misfolding, including polyglutamine (polyQ) diseases. Autophagy is a critical lysosome-mediated degradation pathway, particularly essential in neurons, which...

B. Tedesco, M. Chierichetti, R. Cristofani et al. · Journal of Huntington's Dise... · 0 citations

Open access Sep 2026

A Human Neuronal Cell Model of Endogenous TDP-43 A315T Reveals Altered Protein Dynamics and Disease-Relevant Cellular Dysfunction

A physiologically relevant human model is established that separates early TDP-43 toxic gain-of-function from basal loss-of-function from basal loss-of-function while providing a robust platform for investigating TDP-43 biology and accelerating therapeutic discovery in ALS.

M. Cicardi, Sara Antonini, Ivette Martorell Serra et al. · bioRxiv · 0 citations

Open access Aug 2026

Characterization of the frameshift c.515dupC knock-in mouse model of HSPB8-associated myopathy (MFM13) and evaluation of Trehalose as autophagy-modulating therapy

These findings support the value of preclinical models for translational studies, and autophagy enhancement as a potential therapeutic strategy for HSPB8-related myopathy, and evaluated trehalose, a natural disaccharide that induces HSPB8 and enhances autophagy.

Alyaa Shmara, Lan Weiss, A. Gromova et al. · bioRxiv · 0 citations