title: Strategies to regenerate hair cells: identification of progenitors and critical genes.
abstract: Deafness commonly results from a lesion of the sensory cells and/or of the neurons of the auditory part of the inner ear. There are currently no treatments designed to halt or reverse the progression of hearing loss. A key goal in developing therapy for sensorineural deafness is the identification of strategies to replace lost hair cells. In amphibians and birds, a spontaneous post-injury regeneration of all inner ear sensory hair cells occurs. In contrast, in the mammalian cochlea, hair cells are only produced during embryogenesis. Many studies have been carried out in order to demonstrate the persistence of endogenous progenitors. The present review is first focused on the occurrence of spontaneous supernumerary hair cells and on nestin positive precursors found in the organ of Corti. A second approach to regenerating hair cells would be to find genes essential for their differentiation. This review will also focus on critical genes for embryonic hair cell formation such as the cell cycle related proteins, the Atoh1 gene and the Notch signaling pathway. Understanding mechanisms that underlie hair cell production is an essential prerequisite to defining therapeutic strategies to regenerate hair cells in the mature inner ear.
authors.affiliation: Center for Cellular and Molecular Neurosciences, Developmental Neurobiology Unit, University of Liege, 4000 Liege, Belgium.
authors.forename: Ingrid
authors.initials: I
authors.lastname: Breuskin
authors.forename: Morgan
authors.initials: M
authors.lastname: Bodson
authors.forename: Nicolas
authors.initials: N
authors.lastname: Thelen
authors.forename: Marc
authors.initials: M
authors.lastname: Thiry
authors.forename: Laurent
authors.initials: L
authors.lastname: Nguyen
authors.forename: Shibeshih
authors.initials: S
authors.lastname: Belachew
authors.forename: Philippe P
authors.initials: PP
authors.lastname: Lefebvre
authors.forename: Brigitte
authors.initials: B
authors.lastname: Malgrange
mesh_terms: D000818:Animals; D051792:Basic Helix-Loop-Helix Proteins / Q000235:genetics / Q000502:physiology; D018797:Cell Cycle Proteins / Q000235:genetics / Q000502:physiology; D002454:Cell Differentiation / Q000235:genetics; D006198:Hair Cells, Auditory / Q000166:cytology / Q000502:physiology*; D006801:Humans; D009925:Organ of Corti / Q000196:embryology / Q000254:growth & development / Q000293:injuries; D051880:Receptors, Notch / Q000235:genetics / Q000502:physiology; D012038:Regeneration / Q000235:genetics* / Q000502:physiology*; D015398:Signal Transduction; D013234:Stem Cells / Q000166:cytology
chemical_list: D051792:Basic Helix-Loop-Helix Proteins; D018797:Cell Cycle Proteins; D051880:Receptors, Notch