Hair cells of the inner ear act as mechanosensors, converting mechanical stimuli to electrical signals transmitted to the brain. Hair cells of the auditory organs respond to sound stimuli for hearing perception; those in the vestibular organs respond to gravity and head movements for perception of balance.
Hair cells are called such because they have actin-rich protrusions, stereocilia, at their apical end. Displacement of stereocilia opens ion channels resulting in depolarization and release of transmitter from synapses at the basal end of the cell to terminals of innervating afferent nerves.
Damage and loss of hair cells are leading causes of hearing and balance disorders, affecting over 40 million people in the US. Hair cells are susceptible to environmental insults, including noise, chemical exposure and accumulated damage during aging. Hair cell loss in humans is irreversible.
Zebrafish lateral line hair cells share many properties with those of the inner ear, and are sensitive to the same types of damaging agents as human cells. However they are able to regenerate hair cells throughout life. The location of the lateral line on the surface of the body allows access for visualization and manipulation.
Nechiporuk, A. and Raible, D.W. (2008). Fgf-dependent primordium organization drives lateral line segmentation in zebrafish. Science, 320:1774-1777.
Owens, K.N., Santos, F., Roberts, B., Linbo, T., Knisely, A.J., Simon, J.A., Rubel, E.W and Raible, D.W. (2008). Identification of genetic and chemical modulators of zebrafish mechanosensory hair cell death. PLoS Genetics, 4: e1000020. PMCID: PMC2265478
Ma, E.Y., Rubel, E.W and Raible, D.W. (2008). Notch signaling regulates the extent of hair cell regeneration in the zebrafish lateral line. J. Neurosci., 28: 2261-2273.
Suli, A., Watson, G.M., Rubel, E.W., Raible, D.W. (2012). Rheotaxis in larval zebrafish is mediated by lateral line mechanosensory hair cells. PLoS One, 7:e29727. PMCID:PMC3281009
Hailey, D.W., Roberts, B., Owens, K.N., Stewart, A.K., Linbo, T., Pujol, R., Alper, S.L., Rubel, E.W, Raible, D.W. (2012). Loss Slc4a1b Chloride/Bicarbonate Exchanger Function Protects Mechanosensory Hair Cells from Aminoglycoside Damage in the Zebrafish Mutant persephone. PLoS Genet 8(10): e1002971. PMCID: PMC3469417
Mackenzie, S.M. and Raible, D.W. (2012). Proliferative regeneration of zebrafish lateral line hair cells after different ototoxic insults. PLoS One, 7: e47257. PMCID: PMC3475690
Esterberg, R., Hailey, D.W., Coffin, A.B., Raible, D.W. and Rubel, E.W. (2013). Disruption of intracellular calcium is integral to aminoglycoside-induced hair cell death. J Neurosci., 33: 7513-7525. PMCID: PMC3703319
Coffin, A.B., Rubel, E.W. and Raible, D.W. (2013). Bax, Bcl2, and p53 Differentially Regulate Neomycin- and Gentamicin-Induced Hair Cell Death in the Zebrafish Lateral Line JARO,14: 645-659. PMCID: PMC3767879
Suli, A., Guler, A.D., Raible D.W. and Kimelman, D. (2014). Targeted gene expression system using the Tryptophan Repressor in zebrafish shows no silencing in subsequent generations. Development, 141:1167-1174. PMCID: PMC3929415
Stawicki, T.M., Owens, K.N., Linbo, T., Reinhart, K.E., Rubel, EW, Raible, D.W. (2014). The zebrafish merovingian mutant reveals a role for pH regulation in hair cell toxicity and function. Disease Models Mech., 7:847-56.
Esterberg, R., Hailey, D.W., Rubel, EW, and Raible, D.W. (2014). ER-mitochondrial calcium flow underlies vulnerability of mechanosensory hair cells to damage. J. Neurosci., 34:9703-9719. PMCID: PMC4099547
Thomas, E.D., Cruz, I.A., Hailey, D.W., and Raible D.W. (2015). There and back again: Development and regeneration of the zebrafish lateral line system. Wiley Interdiscip Rev Dev Biol. 4:1-16. PMCID: PMC4268111
Cruz, I.A., Kappedal, R., Mackenzie, S.M., Hailey, D.W., Hoffman, T.L., Schilling, T.F. and Raible, D.W. (2015). Robust regeneration of adult zebrafish lateral line hair cells reflects continued precursor pool maintenance. Dev. Biol., 402: 229-238. PMCID: PMC4450121
Suli, A., Pujol, R., Cunningham, D.E., Hailey, D.W., Prendergast, A., Rubel, E.W. and Raible, D.W. (2016). Innervation Regulates Synaptic Ribbons in Lateral Line Mechanosensory Hair Cells. J Cell Sci. 129:2250-60. PMCID: PMC4920245
Stawicki TM, Hernandez L, Esterberg R, Linbo T, Owens KN, Shah AN, Thapa N, Roberts B, Moens CB, Rubel, E.W. and Raible, D.W. (2016). Cilia genes play differing roles in aminoglycoside-induced hair cell death in zebrafish. G3, 6:2225-35. PMCID: PMC4938675
Esterberg R, Linbo T, Pickett SB, WuP, Ou HC, Rubel, E.W. and Raible, D.W. (2016). Mitochondrial calcium uptake underlies ROS generation during aminoglycoside-induced hair cell death. J. Clinical Invest., 126:3556-66. PMCID: PMC5004972
Hailey, DW, Esterberg R, Linbo T, Rubel, E.W. and Raible, D.W. (2017). Delivery of aminoglycoside antibiotics to the lysosomes predicts their toxicity in mechanosensory hair cells. J. Clinical Invest. 127(2):472-486.
Sebe, J., Cho, S., Sheets, L., Rutherford, M., von Gersdorff, H., and Raible, D.W. (2017). Ca2+ permeable AMPARs mediate glutamatergic transmission and excitotoxic damage at the hair cell afferent synapse. J. Neurosci., 37:6162-6175.
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