title: Proteostasis impairment in ALS.
abstract: In physiological conditions the maintenance of the cellular proteome is a prerequisite for optimal cell functioning and cell survival. Additionally, cells need to constantly sense and adapt to their changing environment and associated stressors. Cells achieve this via a set of molecular chaperones, protein clearance pathways as well as stress-associated signaling networks which work together to prevent protein misfolding, its aggregation and accumulation in subcellular compartments. These processes together form the proteostasis network which helps in maintaining cellular proteostasis. Imbalance or impairment in this processes is directly linked to ageing associated disorders such as diabetes, cancer, stroke, metabolic disorders, pulmonary fibrosis, inflammation and neurodegenerative diseases. In this review, we provide insights into the proteostasis process and how its failure governs neurodegenerative disorders with a special focus on Amyotrophic lateral sclerosis (ALS). This article is part of a Special Issue entitled SI:ER stress.
authors.affiliation: Institute of Cell Biology, University of Bern, Baltzerstrasse 4, CH-3012 Bern, Switzerland; Graduate School for Cellular and Biomedical Sciences, University of Bern, CH-3012 Bern, Switzerland.
authors.forename: Céline
authors.initials: C
authors.lastname: Ruegsegger
authors.affiliation: Institute of Cell Biology, University of Bern, Baltzerstrasse 4, CH-3012 Bern, Switzerland. Electronic address: smita.saxena@izb.unibe.ch.
authors.forename: Smita
authors.initials: S
authors.lastname: Saxena
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D000818:Animals; D001343:Autophagy / Q000502:physiology; D059865:Endoplasmic Reticulum Stress / Q000502:physiology; D006801:Humans; D057165:Proteostasis Deficiencies / Q000150:complications*; D015398:Signal Transduction / Q000502:physiology
keywords: ALS; Autophagy; ER stress; Motoneuron; Neurodegeneration; Proteasome; Proteostasis