title: The role of post-translational modifications in hearing and deafness.
abstract: Post-translational modifications (PTMs) are key molecular events that modify proteins after their synthesis and modulate their ultimate functional properties by affecting their stability, localisation, interaction potential or activity. These chemical changes expand the size of the proteome adding diversity to the molecular pathways governing the biological outcome of cells. PTMs are, thus, crucial in regulating a variety of cellular processes such as apoptosis, proliferation and differentiation and have been shown to be instrumental during embryonic development. In addition, alterations in protein PTMs have been implicated in the pathogenesis of many human diseases, including deafness. In this review, we summarize the recent progress made in understanding the roles of PTMs during cochlear development, with particular emphasis on the enzymes driving protein phosphorylation, acetylation, methylation, glycosylation, ubiquitination and SUMOylation. We also discuss how these enzymes may contribute to hearing impairment and deafness.
authors.affiliation: Developmental Neurobiology Unit, GIGA-Neurosciences, University of Liège, Quartier Hôpital (CHU), Avenue Hippocrate 15, Tour 4, 1er étage, Bât. B36, 4000, Liège, Belgium.
authors.forename: Susana
authors.initials: S
authors.lastname: Mateo Sánchez
authors.affiliation: Developmental Neurobiology Unit, GIGA-Neurosciences, University of Liège, Quartier Hôpital (CHU), Avenue Hippocrate 15, Tour 4, 1er étage, Bât. B36, 4000, Liège, Belgium.
authors.forename: Stephen D
authors.initials: SD
authors.lastname: Freeman
authors.affiliation: Developmental Neurobiology Unit, GIGA-Neurosciences, University of Liège, Quartier Hôpital (CHU), Avenue Hippocrate 15, Tour 4, 1er étage, Bât. B36, 4000, Liège, Belgium.
authors.forename: Laurence
authors.initials: L
authors.lastname: Delacroix
authors.affiliation: Developmental Neurobiology Unit, GIGA-Neurosciences, University of Liège, Quartier Hôpital (CHU), Avenue Hippocrate 15, Tour 4, 1er étage, Bât. B36, 4000, Liège, Belgium. bmalgrange@ulg.ac.be.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Malgrange
mesh_terms: D000818:Animals; D003051:Cochlea / Q000254:growth & development / Q000378:metabolism; D003638:Deafness / Q000378:metabolism / Q000473:pathology*; D006309:Hearing / Q000502:physiology*; D006657:Histones / Q000378:metabolism; D006801:Humans; D011304:Presbycusis / Q000378:metabolism / Q000473:pathology; D011499:Protein Processing, Post-Translational
chemical_list: D006657:Histones
keywords: Cochlea; Development; Inner ear; Ototoxicity; Post-translational modifications