Search Results - "Gopal, Suhasini R"
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Modeling and Preventing Progressive Hearing Loss in Usher Syndrome III
Published in Scientific reports (18-10-2017)“…Usher syndrome type III (USH3) characterized by progressive loss of vision and hearing is caused by mutations in the clarin-1 gene ( CLRN1 ). Clrn1 knockout…”
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Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function
Published in EMBO molecular medicine (01-09-2019)“…Hearing relies on mechanically gated ion channels present in the actin‐rich stereocilia bundles at the apical surface of cochlear hair cells. Our knowledge of…”
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Inhibition of Mitochondrial Division Attenuates Cisplatin-Induced Toxicity in the Neuromast Hair Cells
Published in Frontiers in cellular neuroscience (12-12-2017)“…Cisplatin and other related platinum antineoplastic drugs are commonly used in the treatment of a variety of cancers in both adults and children but are often…”
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Unconventional secretory pathway activation restores hair cell mechanotransduction in an USH3A model
Published in Proceedings of the National Academy of Sciences - PNAS (28-05-2019)“…The pathogenic variant c.144T>G (p.N48K) in the clarin1 gene (CLRN1) results in progressive loss of vision and hearing in Usher syndrome IIIA (USH3A) patients…”
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A small molecule mitigates hearing loss in a mouse model of Usher syndrome III
Published in Nature chemical biology (01-06-2016)“…A small molecule identified from high-throughput screening and optimization specifically stabilizes a disease-linked CLRN1 protein, delivering it to its site…”
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Zebrafish Models for the Mechanosensory Hair Cell Dysfunction in Usher Syndrome 3 Reveal That Clarin-1 Is an Essential Hair Bundle Protein
Published in The Journal of neuroscience (15-07-2015)“…Usher syndrome type III (USH3) is characterized by progressive loss of hearing and vision, and varying degrees of vestibular dysfunction. It is caused by…”
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Noddy, a mouse harboring a missense mutation in protocadherin-15, reveals the impact of disrupting a critical interaction site between tip-link cadherins in inner ear hair cells
Published in The Journal of neuroscience (06-03-2013)“…In hair cells of the inner ear, sound or head movement increases tension in fine filaments termed tip links, which in turn convey force to mechanosensitive ion…”
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