title: Degradation of the Intermediate Filament Family by Gigaxonin.
abstract: Intermediate filament turnover is a highly dynamic process required to maintain tissue integrity and is implicated in degenerative and regenerative processes. Despite these essential roles, little is known about the mechanisms that cause the degradation of intermediate filaments. Nevertheless, the last decade has seen the emergence of the ubiquitin proteasome system, in particular E3 ubiquitin ligases, as important regulators. Here, we will focus on the first identified factor controlling the degradation of the entire intermediate filament family, the gigaxonin-E3 ligase. We will present the scientific achievements and the methodologies to study gigaxonin and its crucial role in intermediate filament turnover.
authors.affiliation: Atip-Avenir Team, Montpellier, France; INM Inserm U1051, Université de Montpellier, Montpellier, France. Electronic address: pascale.bomont@inserm.fr.
authors.forename: Pascale
authors.initials: P
authors.lastname: Bomont
mesh_terms: D000818:Animals; D003598:Cytoskeletal Proteins / Q000502:physiology*; D006801:Humans; D007382:Intermediate Filaments / Q000378:metabolism*; D059748:Proteolysis
chemical_list: D003598:Cytoskeletal Proteins; C501171:Gan protein, mouse
keywords: BTB-Kelch protein; E3 ligase; Giant axonal neuropathy; Gigaxonin; Intermediate filament degradation; Neurodegeneration; Neurofilament; Vimentin