title: The mechanotransduction machinery of hair cells.
abstract: Mechanotransduction, the conversion of mechanical force into an electrochemical signal, allows living organisms to detect touch, hear, register movement and gravity, and sense changes in cell volume and shape. Hair cells in the vertebrate inner ear are mechanoreceptor cells specialized for the detection of sound and head movement. Each hair cell contains, at the apical surface, rows of stereocilia that are connected by extracellular filaments to form an exquisitely organized bundle. Mechanotransduction channels, localized near the tips of the stereocilia, are gated by the gating spring, an elastic element that is stretched upon stereocilia deflection and mediates rapid channel opening. Components of the mechanotransduction machinery in hair cells have been identified and several are encoded by genes linked to deafness in humans, which indicates that defects in the mechanotransduction machinery are the underlying cause of some forms of hearing impairment.
authors.affiliation: Department of Cell Biology, Institute for Childhood and Neglected Disease, Scripps Research Institute, La Jolla, CA 92037, USA.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Grillet
authors.forename: Piotr
authors.initials: P
authors.lastname: Kazmierczak
authors.forename: Wei
authors.initials: W
authors.lastname: Xiong
authors.forename: Martin
authors.initials: M
authors.lastname: Schwander
authors.forename: Anna
authors.initials: A
authors.lastname: Reynolds
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Sakaguchi
authors.forename: Joshua
authors.initials: J
authors.lastname: Tokita
authors.forename: Bechara
authors.initials: B
authors.lastname: Kachar
authors.forename: Ulrich
authors.initials: U
authors.lastname: Müller
mesh_terms: D000818:Animals; D000070585:Cadherin Related Proteins; D015820:Cadherins / Q000502:physiology / Q000648:ultrastructure; D017173:Caenorhabditis elegans / Q000502:physiology; D002352:Carrier Proteins / Q000502:physiology / Q000648:ultrastructure; D018797:Cell Cycle Proteins; D003598:Cytoskeletal Proteins; D006198:Hair Cells, Auditory / Q000502:physiology* / Q000648:ultrastructure; D006309:Hearing / Q000502:physiology; D006319:Hearing Loss, Sensorineural / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D015640:Ion Channel Gating / Q000502:physiology; D008465:Mechanoreceptors / Q000502:physiology; D040542:Mechanotransduction, Cellular / Q000502:physiology*; D051379:Mice; D018345:Mice, Knockout; D008818:Mice, Neurologic Mutants; D016253:Microscopy, Immunoelectron; D025941:Protein Interaction Mapping; D011498:Protein Precursors / Q000502:physiology / Q000648:ultrastructure; D014110:Touch / Q000502:physiology
chemical_list: C418976:CDHR15 protein, mouse; D000070585:Cadherin Related Proteins; D015820:Cadherins; D002352:Carrier Proteins; C499091:Cdh23 protein, mouse; D018797:Cell Cycle Proteins; D003598:Cytoskeletal Proteins; D011498:Protein Precursors; C492262:Ush1c protein, mouse