Harmonin mutations cause mechanotransduction defects in cochlear hair cells.
about
Integrating the biophysical and molecular mechanisms of auditory hair cell mechanotransductionIntestinal brush border assembly driven by protocadherin-based intermicrovillar adhesion.Review series: The cell biology of hearingSensing sound: molecules that orchestrate mechanotransduction by hair cellsStructures of usher syndrome 1 proteins and their complexesThe structure of the harmonin/sans complex reveals an unexpected interaction mode of the two Usher syndrome proteinsVoltage-Gated Cav1 Channels in Disorders of Vision and HearingHarmonin (Ush1c) is required in zebrafish Müller glial cells for photoreceptor synaptic development and functionMyosin VIIa and sans localization at stereocilia upper tip-link density implicates these Usher syndrome proteins in mechanotransductionRegulation of PCDH15 function in mechanosensory hair cells by alternative splicing of the cytoplasmic domainTMHS is an integral component of the mechanotransduction machinery of cochlear hair cellsMurine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function.The murine catecholamine methyltransferase mTOMT is essential for mechanotransduction by cochlear hair cells.Hair cells use active zones with different voltage dependence of Ca2+ influx to decompose sounds into complementary neural codes.Usher syndrome: Hearing loss, retinal degeneration and associated abnormalities.The physiology of mechanoelectrical transduction channels in hearing.Genetics of auditory mechano-electrical transduction.Rescue of hearing and vestibular function by antisense oligonucleotides in a mouse model of human deafness.Molecular remodeling of tip links underlies mechanosensory regeneration in auditory hair cellsLocalization of Usher 1 proteins to the photoreceptor calyceal processes, which are absent from mice.Calcium signaling in the cochlea - Molecular mechanisms and physiopathological implications.Deafness and retinal degeneration in a novel USH1C knock-in mouse modelCadherin-23, myosin VIIa and harmonin, encoded by Usher syndrome type I genes, form a ternary complex and interact with membrane phospholipids.Ush1c gene expression levels in the ear and eye suggest different roles for Ush1c in neurosensory organs in a new Ush1c knockout mouseLarge protein assemblies formed by multivalent interactions between cadherin23 and harmonin suggest a stable anchorage structure at the tip link of stereocilia.Using injectoporation to deliver genes to mechanosensory hair cellsSubunit determination of the conductance of hair-cell mechanotransducer channelsMechanosensory hair cells express two molecularly distinct mechanotransduction channels.Biochemical characterization of native Usher protein complexes from a vesicular subfraction of tracheal epithelial cells.Combination of NMR spectroscopy and X-ray crystallography offers unique advantages for elucidation of the structural basis of protein complex assembly.Tip links in hair cells: molecular composition and role in hearing loss.Targeting of the hair cell proteins cadherin 23, harmonin, myosin XVa, espin, and prestin in an epithelial cell model.Improved biolistic transfection of hair cells.Exome sequencing identifies a founder frameshift mutation in an alternative exon of USH1C as the cause of autosomal recessive retinitis pigmentosa with late-onset hearing loss.Gene therapy restores auditory and vestibular function in a mouse model of Usher syndrome type 1c.Coupling of the mechanotransduction machinery and F-actin polymerization in the cochlear hair bundlesPermeation properties of the hair cell mechanotransducer channel provide insight into its molecular structureEzrin-anchored protein kinase A coordinates phosphorylation-dependent disassembly of a NHERF1 ternary complex to regulate hormone-sensitive phosphate transportCilia-Associated Genes Play Differing Roles in Aminoglycoside-Induced Hair Cell Death in Zebrafish.Usher proteins in inner ear structure and function
P2860
Q22337239-FADFEDC9-4835-4CBD-89D3-11D33AAEDCC3Q24294411-335AD481-17E8-4263-813C-931C76642D9AQ24601353-DD0894C0-4BAA-4EB8-92E0-872A31B62BEDQ26866077-5A28D7BA-4061-4F20-AF7C-88A5791408D2Q27023615-4EAE0018-4295-4D3A-9EC6-D5A5A4250629Q27659663-8E4D4F63-6911-41F9-A79B-FBAD003AFC34Q28084651-3DFDA593-64C8-4C3A-8A16-54AA45C2EAECQ28243260-E2B06495-B722-4AF0-B0BC-90B600E79ACCQ28569812-CAEB0C57-A364-405F-8BD2-026A36DF5C7FQ28590789-9D606FCD-943D-46A0-924A-6FCDC4BE4CD7Q28593852-4FD10CB4-9FF2-4318-8523-D04CD51EF10EQ29347270-6D0EED22-88D6-44F8-94B0-AC545EE40985Q30354602-744DFEEE-08E1-4192-8A46-2D0046411DCAQ30363786-E87D34C7-3108-470F-9AC3-935741AE4BB9Q30389509-0184DF82-B7CE-4851-88E9-59961F9C52B8Q30408022-897BE73A-49A3-41C2-9514-9261F6F691C5Q30421422-4B31DC38-FAFE-43C7-A2BF-6C417858D397Q30450036-179267B1-CFEB-41D8-94D2-A8AE406688CEQ30453450-92F2C669-8D01-4F5C-8AB8-46E137AAFA07Q30455456-2CA084D6-785E-4FA8-BEFF-FC8B5F5C5ADFQ30465350-AEC3F3A6-413D-4B8F-A83A-D393FAE714C1Q30476608-FAF8DDB1-EA3B-4BC9-9C56-17B3A833AEB7Q30479852-B29D3162-F844-442C-BFEE-2C113961512CQ30495605-122AC678-B276-475A-9ED9-57F616065EF2Q30525334-551787BF-82A3-4B4A-A9F1-8E0F5B884B62Q30601270-4F4B745F-09D3-4573-9D79-3969C0D4122BQ30619961-9319301F-5529-45E4-AEB3-EB67C27F445EQ30833261-72372DFD-F566-49AD-8242-9F20FCCBB875Q33745351-D3C94272-2396-4E16-9E47-4BC9BBDE4790Q33819825-18DF9B3F-2BD3-4DC8-9275-219858E64B79Q34066652-54B02501-E699-4757-9A37-08B4852CD45DQ34340843-B58EE927-CA20-4540-A9A0-17ED7FD9B37BQ34437737-F4146C7D-A4F3-4CEB-A7E2-74C58BFA360DQ34516898-F14271DD-5F5B-437D-AFBF-46A733E52187Q34550660-1D6BCE44-C5F9-45FB-A9BE-EFBAAF0BF109Q35534680-CB15DFA2-4F9B-4A78-BFEB-CAF3CF34A169Q35994751-60EB87AE-307E-486C-9987-0E815532A1CFQ36097909-F8214DC3-1D7A-4081-987F-EDB870D63DA0Q37079612-5BF3C811-B221-4394-B091-3DB7D473708EQ37346003-80A43967-6F32-4C5A-836A-183A08F90B15
P2860
Harmonin mutations cause mechanotransduction defects in cochlear hair cells.
description
2009 nî lūn-bûn
@nan
2009 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
2009 թվականի մայիսին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
name
Harmonin mutations cause mechanotransduction defects in cochlear hair cells.
@ast
Harmonin mutations cause mechanotransduction defects in cochlear hair cells.
@en
type
label
Harmonin mutations cause mechanotransduction defects in cochlear hair cells.
@ast
Harmonin mutations cause mechanotransduction defects in cochlear hair cells.
@en
prefLabel
Harmonin mutations cause mechanotransduction defects in cochlear hair cells.
@ast
Harmonin mutations cause mechanotransduction defects in cochlear hair cells.
@en
P2093
P2860
P921
P1433
P1476
Harmonin mutations cause mechanotransduction defects in cochlear hair cells
@en
P2093
Anna Reynolds
Anna Sczaniecka
Bechara Kachar
David Williams
Edith Hintermann
Martin Schwander
Nicolas Grillet
Peter G Gillespie
Piotr Kazmierczak
Rachel A Dumont
P2860
P304
P356
10.1016/J.NEURON.2009.04.006
P407
P577
2009-05-01T00:00:00Z