Proteostasis impairment in ALS.

Ruegsegger, Céline; Saxena, Smita (2016). Proteostasis impairment in ALS. Brain research, 1648(Pt B), pp. 571-579. Elsevier 10.1016/j.brainres.2016.03.032

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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).

Item Type:

Journal Article (Review Article)

Division/Institute:

08 Faculty of Science > Department of Biology > Institute of Cell Biology

Graduate School:

Graduate School for Cellular and Biomedical Sciences (GCB)

UniBE Contributor:

Ruegsegger, Céline and Saxena, Smita

Subjects:

500 Science > 570 Life sciences; biology

ISSN:

0006-8993

Publisher:

Elsevier

Language:

English

Submitter:

Prof. Smita Saxena

Date Deposited:

01 Jun 2016 13:56

Last Modified:

28 Sep 2016 01:34

Publisher DOI:

10.1016/j.brainres.2016.03.032

PubMed ID:

27033833

BORIS DOI:

10.7892/boris.80838

URI:

https://boris.unibe.ch/id/eprint/80838

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