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Unconventional secretory pathway activation restores hair cell mechanotransduction in an USH3A model

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. CLRN1 is predicted to be an essential protein in hair bundles, the mechanosensory structure of hair cells critical for hearing and balanc...

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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:Proc Natl Acad Sci U S A
Prif Awduron: Gopal, Suhasini R., Lee, Yvonne T., Stepanyan, Ruben, McDermott, Brian M., Alagramam, Kumar N.
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: National Academy of Sciences 2019
Pynciau:
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC6561152/
https://ncbi.nlm.nih.gov/pubmed/31097578
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1817500116
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