9/25/2023 0 Comments Has many complex proteins and so have a robust system for heat-shock protein assisted folding![]() HSPB8 is expressed both in motoneuron and muscle cells, which are both targets of misfolded protein toxicity in MNDs. The small heat shock protein B8 (HSPB8) is a chaperone induced by harmful events, like proteasome inhibition. To prevent proteotoxic stresses detrimental to cells, misfolded and/or aggregated proteins must be rapidly removed by the protein quality control (PQC) system. Notably, some of these proteins accumulate into aggregates also in sporadic ALS (sALS) even if not mutated. In familial ALS (fALS) and in SBMA specific gene mutations lead to the production of neurotoxic proteins or peptides prone to misfold, which then accumulate in form of aggregates. 4Centro Interuniversitario sulle Malattie Neurodegenerative, Università degli Studi di Firenze, Roma Tor Vergata, Milano, ItalyĪmyotrophic lateral sclerosis (ALS) and spinal and bulbar muscular atrophy (SBMA) are two motoneuron diseases (MNDs) characterized by aberrant protein behavior in affected cells.Mondino National Neurological Institute, Pavia, Italy 2Dipartimento di Scienze Biomediche, Metaboliche e Neuroscienze, Università di Modena e Reggio Emilia, Modena, Italy.1Dipartimento di Scienze Farmacologiche e Biomolecolari (DiSFeB), Centro di Eccellenza sulle Malattie Neurodegenerative, Università degli Studi di Milano, Milano, Italy.Cicardi 1 Marco Meroni 1 Veronica Ferrari 1 Giulia Vezzoli 1 Barbara Tedesco 1 Elio Messi 1 Margherita Piccolella 1 Serena Carra 2 Valeria Crippa 1,3† Angelo Poletti 1,4*† ![]() Paola Rusmini 1† Riccardo Cristofani 1† Mariarita Galbiati 1 Maria E. ![]()
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