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EnglishMotor neuron diseases (MNDs) are fatal diseases characterized by loss of motor neurons in the brain cortex, in the bulbar region, and/or in the anterior horns of the spinal cord. While generally sporadic, inherited forms linked to mutant genes encoding altered RNA/protein products have also been described. Several different mechanisms have been found altered or dysfunctional in MNDs, like the protein quality control (PQC) system. In this review, we will discuss how the PQC system is affected in two MNDs—spinal and bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS)—and how this affects the clearance of aberrantly folded proteins, which accumulate in motor neurons, inducing dysfunctions and their death. In addition, we will discuss how the PQC system can be targeted to restore proper cell function, enhancing the survival of affected cells in MNDs.
A Crucial Role for the Protein Quality Control System in Motor Neuron Diseases / R. Cristofani, V. Crippa, M.E. Cicardi, B. Tedesco, V. Ferrari, M. Chierichetti, E. Casarotto, M. Piccolella, E. Messi, M. Galbiati, P. Rusmini, A. Poletti. - In: FRONTIERS IN AGING NEUROSCIENCE. - ISSN 1663-4365. - 12(2020 Jul 21).
| Titolo: | A Crucial Role for the Protein Quality Control System in Motor Neuron Diseases |
| Autori: | CRISTOFANI, RICCARDO MARIA (Primo) CRIPPA, VALERIA (Secondo) RUSMINI, PAOLA (Penultimo) POLETTI, ANGELO (Ultimo) (Corresponding) |
| Parole Chiave: | motor neuron; ALS; SBMA; MND; protein quality control; CASA complex; HSPB8; BAG3; BAG1; proteasome; autophagy; misfolding; aggregation |
| Settore Scientifico Disciplinare: | Settore BIO/13 - Biologia Applicata Settore BIO/09 - Fisiologia |
| Progetto: | Alternative translation initiation as a novel strategy to block toxicity of the mutant Androgen Receptor in SBMA Motor neuron degeneration in Spinal and Bulbar Muscular Atrophy: molecular approaches to counteract mutant androgen receptor neurotoxicity Selective translation of androgen receptor isoform A to prevent polyQ mediated toxicity in Kennedy’s disease Dai modelli cellulari ai trial clinici: la malattia di Kennedy come paradigma di ricerca traslazionale Colchicine for Amyotrophic Lateral Sclerosis: a phase II, randomized, double blind, placebo controlled, multicenter clinical trial (Co-ALS) Stress granules and proteostasis in motor neurons: towards a mechanistic understanding of ALS (CureALS) Membrane-less organelle pathology in ALS: identification of causes and rescuing factors (MLOMALS) RAN translation of normal and expanded nucleotide repeat containing transcripts to neurotoxic polypetides in neurodegenerative diseases Targeting RAN translation in ALS (Target-RAN) Ruolo protettivo della proteina HSPB8 nelle malattie del motoneurone Upregulation of HSPB8 as potential therapeutic approach in familial and sporadic ALS Interplay between Androgenic/Anabolic Steroid and IGF-1 Signaling in Amyotrophic Lateral Sclerosis The interplay between the “rna/protein quality control system” and “exosomes” as a spreading mechanism in amyotrophic lateral sclerosis (EX_ALS) Selective autophagic response to proteotoxicity in motorneurons and muscle of motor neuron diseases Translating molecular mechanisms into ALS risk and patient's well-being From RNA to Protein toxicity in motorneuron diseases From RNA to Protein toxicity in motorneuron diseases |
| Data di pubblicazione: | 21-lug-2020 |
| Rivista: | |
| Centro di ricerca: | Centro Interdipartimentale di Eccellenza per le Malattie Neurodegenerative CEND |
| Tipologia: | Article (author) |
| Digital Object Identifier (DOI): | http://dx.doi.org/10.3389/fnagi.2020.00191 |
| Appare nelle tipologie: | 01 - Articolo su periodico |
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