Modelling FUSopathies: focus on protein aggregation

Shelkovnikova, Tatyana A. ORCID: https://orcid.org/0000-0003-1367-5309 2013. Modelling FUSopathies: focus on protein aggregation. Biochemical Society Transactions 41 (6) , pp. 1613-1617. 10.1042/BST20130212

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Abstract

The discovery of a causative link between dysfunction of a number of RNA-binding proteins with prion-like domains and the development of certain (neuro)degenerative diseases has completely changed our perception of molecular mechanisms instigating pathological process in these disorders. Irreversible aggregation of these proteins is a crucial pathogenic event delineating a type of proteinopathy. FUS (fused in sarcoma) is a prototypical member of the class, and studies into the causes and consequences of FUSopathies have been instrumental in characterizing the processes leading to deregulation of RNA metabolism in neurodegeneration. In vivo models of FUSopathy have provided critical insights into the mechanisms of FUS toxicity and clues on the role of non-amyloid aggregates, which are hallmarks of these diseases. The present review summarizes the data on FUS aggregation signatures in available model organisms on the basis of overexpression of FUS variants.

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Biosciences
Publisher:	Portland Press
ISSN:	0300-5127
Last Modified:	25 Oct 2022 13:44
URI:	https://orca.cardiff.ac.uk/id/eprint/120668

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