Genomes and Genes
Affiliation: University College London
- Rapid hair cell loss: a mouse model for cochlear lesionsRuth Rebecca Taylor
Centre for Auditory Research, UCL Ear Institute, University College London, 332 Gray s Inn Road, London, WC1X 8EE, UK
J Assoc Res Otolaryngol 9:44-64. 2008..This in vivo protocol provides a robust model in which to obtain extensive OHC loss in the mature cochleae of mice and is a means with which to examine different aspects of cochlear pathology in transgenic or mutant strains...
- Connexin30-mediated intercellular communication plays an essential role in epithelial repair in the cochleaAndrew Forge
Centre for Auditory Research, UCL Ear Institute, London WC1X 8EE, UK
J Cell Sci 126:1703-12. 2013..These observations demonstrate a role for Cx30 and intercellular communication in regulating repair responses in an epithelial tissue...
- β3-integrin is required for differentiation in OC-2 cells derived from mammalian embryonic inner earIvan Brunetta
Centre for Auditory Research, UCL Ear Institute, University College London, London WC1X 8EE, UK
BMC Cell Biol 13:5. 2012..The OC-2 cells are thus a useful model system for testing mechanism of hair cells differentiation...
- Opioid modulation of GABA release in the rat inferior colliculusWalaiporn Tongjaroenbungam
Neuro Behavioural Biology Centre, Mahidol University Salaya Nakom Pathom 73170 Thailand
BMC Neurosci 5:31. 2004....
- The inner ear contains heteromeric channels composed of cx26 and cx30 and deafness-related mutations in cx26 have a dominant negative effect on cx30Andrew Forge
UCL Centre for Auditory Research and Institute of Laryngology and Otology, University College London, London, United Kingdom
Cell Commun Adhes 10:341-6. 2003..The functional disruption caused by some cx26 mutations upon such heteromeric channels may underlie the non-syndromic nature of their effects on hearing...
- Developmental biology. Life after deaf for hair cells?Ruth Taylor
Centre for Auditory Research, UCL Ear Institute, University College London, London WC1X 8EE, UK
Science 307:1056-8. 2005
- Gap junctions in the inner ear: comparison of distribution patterns in different vertebrates and assessement of connexin composition in mammalsAndrew Forge
UCL Centre for Auditory Research and Institute of Laryngology and Otology, University College London, London WC1X 8EE, United Kingdom
J Comp Neurol 467:207-31. 2003..Heteromeric cx26/cx30 connexons may be unique to the inner ear, which could be one factor underlying the non-syndromic character of the deafness caused by mutations in cx26...
- Connexins and gap junctions in the inner earAndrew Forge
UCL Centre for Auditory Research and Institute of Laryngology and Otology, UCL, London, UK
Audiol Neurootol 7:141-5. 2002....
- Apoptotic death of hair cells in mammalian vestibular sensory epitheliaA Forge
Institute of Laryngology and Otology, University College London, 330 332 Gray s Inn Road, London, UK
Hear Res 139:97-115. 2000..Explant cultures of mature vestibular organs may be, therefore, a useful model system for examining putative hair cell protecting agents...
- Hair cell recovery in the vestibular sensory epithelia of mature guinea pigsA Forge
Institute of Laryngology and Otology, University College London, United Kingdom
J Comp Neurol 397:69-88. 1998..There was also no evidence of any hair cell recovery in the organ of Corti. Thus, there appear to be limitations on the capacity for spontaneous replacement of lost hair cells in the mammalian inner ear...
- Mutations in the gene for connexin 26 (GJB2) that cause hearing loss have a dominant negative effect on connexin 30Nerissa K Marziano
UCL Centre for Auditory Research and Institute of Laryngology and Otology, 330 332 Gray s Inn Road, London WC1X 8EE, UK
Hum Mol Genet 12:805-12. 2003..Disruption of these heteromeric channels by certain mutations may underlie the non-syndromic nature of the deafness...
- Postnatal maturation of the organ of Corti in gerbils: morphology and physiological responsesM Souter
Institute of Laryngology and Otology, University College London Medical School, England
J Comp Neurol 386:635-51. 1997....
- Gap junctions and connexins in the inner ear: their roles in homeostasis and deafnessRegina Nickel
Centre for Auditory Research, UCL Ear Institute, University College London, London, UK
Curr Opin Otolaryngol Head Neck Surg 16:452-7. 2008..In this review, we discuss current ideas about the roles of gap junctions in the inner ear and the implications of connexin mutations on auditory function...
- Molecular and functional characterization of gap junctions in the avian inner earRegina Nickel
Centre for Auditory Research, The Ear Institute, University College London, London WC1X 8EE, United Kingdom
J Neurosci 26:6190-9. 2006..Furthermore, it demonstrates asymmetric GJC within the supporting cell population of the auditory sensory epithelium, which might mediate potassium cycling and/or intercellular signaling...
- Alström Syndrome protein ALMS1 localizes to basal bodies of cochlear hair cells and regulates cilium-dependent planar cell polarityDaniel Jagger
UCL Ear Institute, University College London, 332 Gray s Inn Road, London WC1X 8EE, UK
Hum Mol Genet 20:466-81. 2011....
- Compartmentalized and signal-selective gap junctional coupling in the hearing cochleaDaniel J Jagger
Centre for Auditory Research, UCL Ear Institute, University College London, London WC1X 8EE, United Kingdom
J Neurosci 26:1260-8. 2006..Furthermore, they suggest that separate medial and lateral buffering compartments exist in the hearing cochlea, which are individually dedicated to the homeostasis of inner hair cells and outer hair cells...
- The molecular architecture of the inner earAndrew Forge
UCL Centre for Auditory Research and Institute of Laryngology and Otology, University College London, UK
Br Med Bull 63:5-24. 2002..This work is providing new insights into how the tissues of the inner ear are built to perform their tasks, and into the pathogenesis of a range of inner ear disorders...
- Hair cell regeneration in sensory epithelia from the inner ear of a urodele amphibianRuth R Taylor
UCL Centre for Auditory Research, University College London, London WC1X 8EE, United Kingdom
J Comp Neurol 484:105-20. 2005..A similar mechanism has been proposed for the hair cell recovery phenomenon observed in the vestibular organs of mammals...
- Development of gap junctional intercellular communication within the lateral wall of the rat cochleaJ J Kelly
UCL Ear Institute, University College London, 332 Gray s Inn Road, London WC1X8EE, UK
Neuroscience 180:360-9. 2011..These data suggest that lateral wall function matures several days in advance of hearing onset, and provide anatomical evidence of the existence of a putative K+ recirculation pathway within the cochlear lateral wall...
- Intercellular junctional maturation in the stria vascularis: possible association with onset and rise of endocochlear potentialM Souter
Institute of Laryngology and Otology, University College London Medical School, UK
Hear Res 119:81-95. 1998....
- Patterns of expression of Bardet-Biedl syndrome proteins in the mammalian cochlea suggest noncentrosomal functionsHelen L May-Simera
Institute of Child Health, University College London, London WC1N1EH, United Kingdom
J Comp Neurol 514:174-88. 2009..In summary, our findings indicate that BBS proteins adopt a range of cellular distributions in vivo, not restricted to the centrosome or cilium, and so broaden the possible underlying pathomechanisms of the disease...
- Arp2/3 complex-mediated actin polymerisation occurs on specific pre-existing networks in cells and requires spatial restriction to sustain functional lamellipod extensionD Shao
Division of Cell Biology, UCL Institute of Ophthalmology, London, United Kingdom
Cell Motil Cytoskeleton 63:395-414. 2006....
- A congenital activating mutant of WASp causes altered plasma membrane topography and adhesion under flow in lymphocytesSiobhan O Burns
Molecular Immunology Unit, Centre for Immunodeficiency, Institute of Child Health, University College London, London, UK
Blood 115:5355-65. 2010..Together, our results demonstrate that WASp(I294T) significantly affects lymphocyte membrane topography and L-selectin-dependent adhesion, which may be linked to defective hematopoiesis and leukocyte function in affected patients...
- Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebratesAlison J Ross
Molecular Medicine Unit, Institute of Child Health, University College London, WC1N 1EH, UK
Nat Genet 37:1135-40. 2005..We also show that Vangl2 localizes to the basal body and axoneme of ciliated cells, a pattern reminiscent of that of the BBS proteins. These data suggest that cilia are intrinsically involved in PCP processes...
- MicroRNAs and regeneration: Let-7 members as potential regulators of dedifferentiation in lens and inner ear hair cell regeneration of the adult newtPanagiotis A Tsonis
Department of Biology and Center for Tissue Regeneration and Engineering, University of Dayton, Dayton, OH 45469 2320, USA
Biochem Biophys Res Commun 362:940-5. 2007..This is the first report to correlate expression of miRNAs as novel regulators of vertebrate regeneration, alluding to a novel mechanism whereby transdifferentiation occurs...
- Claudin 14 knockout mice, a model for autosomal recessive deafness DFNB29, are deaf due to cochlear hair cell degenerationTamar Ben-Yosef
Laboratory of Molecular Genetics, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, MD 20850, USA
Hum Mol Genet 12:2049-61. 2003..Our data suggest that the TJ complex at the apex of the reticular lamina requires claudin 14 as a cation-restrictive barrier to maintain the proper ionic composition of the fluid surrounding the basolateral surface of outer hair cells...
- The existence of opioid receptors in the cochlea of guinea pigsNopporn Jongkamonwiwat
Neuro Behavioural Biology Center, Institute of Science and Technology for Research and Development, Mahidol University, Salaya Campus, Nakornpathom 73170, Thailand
Eur J Neurosci 23:2701-11. 2006....
- Tonotopic variation in the calcium dependence of neurotransmitter release and vesicle pool replenishment at mammalian auditory ribbon synapsesStuart L Johnson
Department of Biomedical Science, University of Sheffield, Sheffield, United Kingdom
J Neurosci 28:7670-8. 2008..Together, our findings provide the first evidence for a tonotopic difference in the properties of the synaptic machinery in mammalian IHCs, which could be essential for fine-tuning their receptor characteristics during sound stimulation...
- Tricellulin is a tight-junction protein necessary for hearingSaima Riazuddin
Section on Human Genetics, Laboratory of Molecular Genetics, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, MD 20850, USA
Am J Hum Genet 79:1040-51. 2006..A wild-type isoform of tricellulin, which lacks this conserved region, is unaffected by the mutant alleles and is hypothesized to be sufficient for structural and functional integrity of epithelial barriers outside the inner ear...
- Asymmetric localization of Vangl2 and Fz3 indicate novel mechanisms for planar cell polarity in mammalsMireille Montcouquiol
Section on Developmental Neuroscience, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland 20892, USA
J Neurosci 26:5265-75. 2006..Together, our data support the idea that protein asymmetry plays an important role in the development of PCP, but the colocalization and interaction of Fz3 and Vangl2 suggests that novel PCP mechanisms exist in vertebrates...
- The presence of opioid receptors in rat inner earNopporn Jongkamonwiwat
Neuro Behavioural Biology Center, Institute of Science and Technology for Research and Development, Mahidol University, 73170 Nakornpathom, Thailand
Hear Res 181:85-93. 2003..These findings are the first report of the presence of all three classical opioid receptors in the inner ear and suggest that these receptors may have both presynaptic and postsynaptic roles...
- Low density of membrane particles in auditory hair cells of lizards and birds suggests an absence of somatic motilityChristine Köppl
Lehrstuhl für Zoologie, Technische Universitat Munchen, 85747 Garching, Germany
J Comp Neurol 479:149-55. 2004..This suggests that nonmammalian hair cells do not possess high densities of motor protein in their membranes and are thus unlikely to be capable of somatic motility...
- The opioid receptors in inner ear of different stages of postnatal ratsPansiri Phansuwan-Pujito
Department of Anatomy, Faculty of Medicine, Srinakharinwirot University, Bangkok 10110, Thailand
Hear Res 184:1-10. 2003..The data provide further evidence that the opioid system may play a role in the development and functioning of the inner ear...