期刊
FRONTIERS IN AGING NEUROSCIENCE
卷 12, 期 -, 页码 -出版社
FRONTIERS MEDIA SA
DOI: 10.3389/fnagi.2020.00191
关键词
motor neuron; protein quality control; CASA complex; HSPB8; BAG3; BAG1
资金
- Fondazione Telethon, Italy [GGP14039, GGP19128]
- Kennedy's disease association (2018 grant)
- Fondazione Cariplo, Italy [2014-0686, 2017_0747]
- Fondazione AriSLA, Italy [ALS_HSPB8]
- Association Francaise contre les Myopathies, France (AFM Telethon) [16406]
- Universita degli Studi di Milano e piano di sviluppo UNIMI-linea B
- Italian Ministry of University and Research (MIUR), PRIN-Progetti di ricerca di interesse nazionale [2015LFPNMN, 2017F2A2C5]
- Fondo per il Finanziamento delle Attivita Base di Ricerca (FFABR
- MIUR)
- Agenzia Italiana del Farmaco (AIFA
- Co_ALS)
- Italian Ministry of Health [GR-2011-02347198]
- Fondazione Regionale per la Ricerca Biomedica (FRRB
- Regione Lombardia, TRANS_ALS) [2015-0023]
- EU Joint Programme-Neurodegenerative Disease Research (JPND) project
- European Union's Horizon 2020 research and innovation program [643417, 01ED1601A]
- Italian Ministry of University and Research (Progetto Dipartimenti di Eccellenza)
- JPND
- Bando Straordinario per Progetti Interdipartimentali (Bando SEED 2019) [TDP-43-iPSC]
- Fondazione AriSLA, Italy (ALS_Granulopathy)
- Fondazione AriSLA, Italy (MLOpathy)
- Fondazione AriSLA, Italy (Target-RAN)
Motor 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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据