Search Results - "Guy Richardson"

Hair-Bundle Links: Genetics as the Gateway to Function by Richardson, Guy P, Petit, Christine

“…Up to five distinct cell-surface specializations interconnect the stereocilia and the kinocilium of the mature hair bundle in some species: kinocilial links,…”
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Linking genes underlying deafness to hair-bundle development and function by Petit, Christine, Richardson, Guy P

“…This review describes how the genetic causes of the many forms of human deafness have provided insight into the molecular mechanisms of auditory transduction…”
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Easi-CRISPR: a robust method for one-step generation of mice carrying conditional and insertion alleles using long ssDNA donors and CRISPR ribonucleoproteins by Quadros, Rolen M, Miura, Hiromi, Harms, Donald W, Akatsuka, Hisako, Sato, Takehito, Aida, Tomomi, Redder, Ronald, Richardson, Guy P, Inagaki, Yutaka, Sakai, Daisuke, Buckley, Shannon M, Seshacharyulu, Parthasarathy, Batra, Surinder K, Behlke, Mark A, Zeiner, Sarah A, Jacobi, Ashley M, Izu, Yayoi, Thoreson, Wallace B, Urness, Lisa D, Mansour, Suzanne L, Ohtsuka, Masato, Gurumurthy, Channabasavaiah B

“…Conditional knockout mice and transgenic mice expressing recombinases, reporters, and inducible transcriptional activators are key for many genetic studies and…”
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Cell-Cell Contact Area Affects Notch Signaling and Notch-Dependent Patterning by Shaya, Oren, Binshtok, Udi, Hersch, Micha, Rivkin, Dmitri, Weinreb, Sheila, Amir-Zilberstein, Liat, Khamaisi, Bassma, Oppenheim, Olya, Desai, Ravi A., Goodyear, Richard J., Richardson, Guy P., Chen, Christopher S., Sprinzak, David

“…During development, cells undergo dramatic changes in their morphology. By affecting contact geometry, these morphological changes could influence cellular…”
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Structure, Function, and Development of the Tectorial Membrane: An Extracellular Matrix Essential for Hearing by Goodyear, Richard J, Richardson, Guy P

“…The tectorial membrane is an extracellular matrix that lies over the apical surface of the auditory epithelia in the inner ears of reptiles, birds, and…”
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How the genetics of deafness illuminates auditory physiology by Richardson, Guy P, de Monvel, Jacques Boutet, Petit, Christine

“…Although the basic principles underlying the function of the peripheral auditory system have been known for many years, the molecules required for hearing have…”
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Mutations in protocadherin 15 and cadherin 23 affect tip links and mechanotransduction in mammalian sensory hair cells by Alagramam, Kumar N, Goodyear, Richard J, Geng, Ruishuang, Furness, David N, van Aken, Alexander F J, Marcotti, Walter, Kros, Corné J, Richardson, Guy P

“…Immunocytochemical studies have shown that protocadherin-15 (PCDH15) and cadherin-23 (CDH23) are associated with tip links, structures thought to gate the…”
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Genetically modified mouse models to help fight COVID-19 by Gurumurthy, Channabasavaiah B., Quadros, Rolen M., Richardson, Guy P., Poluektova, Larisa Y., Mansour, Suzanne L., Ohtsuka, Masato

“…The research community is in a race to understand the molecular mechanisms of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, to…”
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Ultrastructural defects in stereocilia and tectorial membrane in aging mouse and human cochleae by Bullen, Anwen, Forge, Andrew, Wright, Anthony, Richardson, Guy P., Goodyear, Richard J., Taylor, Ruth

“…The aging cochlea is subjected to a number of pathological changes to play a role in the onset of age‐related hearing loss (ARHL). Although ARHL has often been…”
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Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane by Verpy, Elisabeth, Leibovici, Michel, Michalski, Nicolas, Goodyear, Richard J., Houdon, Carine, Weil, Dominique, Richardson, Guy P., Petit, Christine

“…Stereocilin is defective in a recessive form of deafness, DFNB16. We studied the distribution of stereocilin in the developing and mature mouse inner ear and…”
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Author Correction: Sharpened cochlear tuning in a mouse with a genetically modified tectorial membrane by Russell, Ian J., Legan, P. Kevin, Lukashkina, Victoria A., Lukashkin, Andrei N., Goodyear, Richard J., Richardson, Guy P.

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A tectorin-based matrix and planar cell polarity genes are required for normal collagen-fibril orientation in the developing tectorial membrane by Goodyear, Richard J, Lu, Xiaowei, Deans, Michael R, Richardson, Guy P

“…The tectorial membrane is an extracellular structure of the cochlea. It develops on the surface of the auditory epithelium and contains collagen fibrils…”
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Age‐related changes in the biophysical and morphological characteristics of mouse cochlear outer hair cells by Jeng, Jing‐Yi, Johnson, Stuart L., Carlton, Adam J, De Tomasi, Lara, Goodyear, Richard J., De Faveri, Francesca, Furness, David N., Wells, Sara, Brown, Steve D. M., Holley, Matthew C., Richardson, Guy P., Mustapha, Mirna, Bowl, Michael R., Marcotti, Walter

“…Key points Age‐related hearing loss (ARHL) is a very heterogeneous disease, resulting from cellular senescence, genetic predisposition and environmental…”
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A Mutation in PNPT1, Encoding Mitochondrial-RNA-Import Protein PNPase, Causes Hereditary Hearing Loss by VON AMELN, Simon, GENG WANG, BORCK, Guntram, HONG, Jason S, GOODYEAR, Richard J, ABIDI, Omar, NÜRNBERG, Peter, HOFMANN, Kay, RICHARDSON, Guy P, HAMMERSCHMIDT, Matthias, MOSER, Tobias, WOLLNIK, Bernd, BOULOUIZ, Redouane, KOEHLER, Carla M, TEITELL, Michael A, BARAKAT, Abdelhamid, KUBISCH, Christian, RUTHERFORD, Mark A, SMITH, Geoffrey M, YUN LI, POGODA, Hans-Martin, NIIRNBERG, Gudrun, STILLER, Barbara, VOLK, Alexander E

“…A subset of nuclear-encoded RNAs has to be imported into mitochondria for the proper replication and transcription of the mitochondrial genome and, hence, for…”
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A critical period of prehearing spontaneous Ca2+ spiking is required for hair‐bundle maintenance in inner hair cells by Carlton, Adam J, Jeng, Jing‐Yi, Grandi, Fiorella C, De Faveri, Francesca, Ceriani, Federico, De Tomasi, Lara, Underhill, Anna, Johnson, Stuart L, Legan, Kevin P, Kros, Corné J, Richardson, Guy P, Mustapha, Mirna, Marcotti, Walter

“…Sensory‐independent Ca 2+ spiking regulates the development of mammalian sensory systems. In the immature cochlea, inner hair cells (IHCs) fire spontaneous Ca…”
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Sharpened cochlear tuning in a mouse with a genetically modified tectorial membrane by Richardson, Guy P, Goodyear, Richard J, Lukashkina, Victoria A, Russell, Ian J, Legan, P Kevin, Lukashkin, Andrei N

“…Frequency tuning in the cochlea is determined by the passive mechanical properties of the basilar membrane and active feedback from the outer hair cells,…”
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Stereocilin-deficient mice reveal the origin of cochlear waveform distortions by Verpy, Elisabeth, Leibovici, Michel, Houdon, Carine, Goodyear, Richard J, Weil, Dominique, Petit, Christine, Avan, Paul, Hamard, Ghislaine, Hardelin, Jean-Pierre, Richardson, Guy P, Lefèvre, Gaelle M

“…Although the cochlea is an amplifier and a remarkably sensitive and finely tuned detector of sounds, it also produces conspicuous mechanical and electrical…”
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MET currents and otoacoustic emissions from mice with a detached tectorial membrane indicate the extracellular matrix regulates Ca2+ near stereocilia by Jeng, Jing‐Yi, Harasztosi, Csaba, Carlton, Adam J., Corns, Laura F., Marchetta, Philine, Johnson, Stuart L., Goodyear, Richard J., Legan, Kevin P., Rüttiger, Lukas, Richardson, Guy P., Marcotti, Walter

“…Key points The aim was to determine whether detachment of the tectorial membrane (TM) from the organ of Corti in Tecta/Tectb−/− mice affects the biophysical…”
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Porosity Controls Spread of Excitation in Tectorial Membrane Traveling Waves by Sellon, Jonathan B., Ghaffari, Roozbeh, Farrahi, Shirin, Richardson, Guy P., Freeman, Dennis M.

“…Cochlear frequency selectivity plays a key role in our ability to understand speech, and is widely believed to be associated with cochlear amplification…”
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Tectorial membrane travelling waves underlie abnormal hearing in Tectb mutant mice by Freeman, Dennis M, Ghaffari, Roozbeh, Aranyosi, Alexander J, Richardson, Guy P

“…Remarkable sensitivity and exquisite frequency selectivity are hallmarks of mammalian hearing, but their underlying mechanisms remain unclear. Cochlear insults…”
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