Trehalose induces autophagy via lysosomal-mediated TFEB activation in models of motoneuron degeneration

…, ME Cicardi, V Ferrari, G Vezzoli, B Tedesco… - Autophagy, 2019 - Taylor & Francis
Macroautophagy/autophagy, a defense mechanism against aberrant stresses, in neurons
counteracts aggregate-prone misfolded protein toxicity. Autophagy induction might be …

The chaperone-assisted selective autophagy complex dynamics and dysfunctions

B Tedesco, L Vendredy, V Timmerman, A Poletti - Autophagy, 2023 - Taylor & Francis
Each protein must be synthesized with the correct amino acid sequence, folded into its native
structure, and transported to a relevant subcellular location and protein complex. If any of …

[HTML][HTML] The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases

…, ME Cicardi, M Meroni, V Ferrari, B Tedesco… - Cell Stress and …, 2018 - Springer
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two
neurodegenerative diseases in which similar pathogenic mechanisms are involved. Both diseases …

[HTML][HTML] The role of HSPB8, a component of the chaperone-assisted selective autophagy machinery, in cancer

R Cristofani, M Piccolella, V Crippa, B Tedesco… - Cells, 2021 - mdpi.com
The cellular response to cancer-induced stress is one of the major aspects regulating cancer
development and progression. The Heat Shock Protein B8 (HSPB8) is a small chaperone …

[HTML][HTML] The role of the heat shock protein B8 (HSPB8) in motoneuron diseases

…, M Meroni, V Ferrari, G Vezzoli, B Tedesco… - Frontiers in molecular …, 2017 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) and spinal and bulbar muscular atrophy (SBMA) are two
motoneuron diseases (MNDs) characterized by aberrant protein behavior in affected cells. …

[HTML][HTML] Tdp-25 routing to autophagy and proteasome ameliorates its aggregation in amyotrophic lateral sclerosis target cells

…, M Meroni, V Ferrari, G Vezzoli, B Tedesco… - Scientific reports, 2018 - nature.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that primarily affects
motoneurons, while non-neuronal cells may contribute to disease onset and progression. …

[HTML][HTML] Valosin containing protein (VCP): a multistep regulator of autophagy

V Ferrari, R Cristofani, B Tedesco, V Crippa… - International Journal of …, 2022 - mdpi.com
Valosin containing protein (VCP) has emerged as a central protein in the regulation of the
protein quality control (PQC) system. VCP mutations are causative of multisystem …

[HTML][HTML] BAG3 Pro209 mutants associated with myopathy and neuropathy relocate chaperones of the CASA-complex to aggresomes

E Adriaenssens, B Tedesco, L Mediani… - Scientific Reports, 2020 - nature.com
Three missense mutations targeting the same proline 209 (Pro209) codon in the co-chaperone
Bcl2-associated athanogene 3 (BAG3) have been reported to cause distal myopathy, …

[HTML][HTML] Multiple roles of transforming growth factor beta in amyotrophic lateral sclerosis

…, R Cristofani, E Messi, M Piccolella, B Tedesco… - International Journal of …, 2020 - mdpi.com
Transforming growth factor beta (TGFB) is a pleiotropic cytokine known to be dysregulated
in many neurodegenerative disorders and particularly in amyotrophic lateral sclerosis (ALS). …

HSPB8 frameshift mutant aggregates weaken chaperone-assisted selective autophagy in neuromyopathies

B Tedesco, L Vendredy, E Adriaenssens, M Cozzi… - Autophagy, 2023 - Taylor & Francis
Chaperone-assisted selective autophagy (CASA) is a highly selective pathway for the
disposal of misfolding and aggregating proteins. In muscle, CASA assures muscle integrity by …