Grxcr1 Promotes Hair Bundle Development by Destabilizing the Physical Interaction between Harmonin and Sans Usher Syndrome Proteins

Publication date: 30 October 2018Source: Cell Reports, Volume 25, Issue 5Author(s): Bernardo Blanco-Sánchez, Aurélie Clément, Javier Fierro, Sarah Stednitz, Jennifer B. Phillips, Jeremy Wegner, Jennifer M. Panlilio, Judy L. Peirce, Philip Washbourne, Monte WesterfieldSummaryMorphogenesis and mechanoelectrical transduction of the hair cell mechanoreceptor depend on the correct assembly of Usher syndrome (USH) proteins into highly organized macromolecular complexes. Defects in these proteins lead to deafness and vestibular areflexia in USH patients. Mutations in a non-USH protein, glutaredoxin domain-containing cysteine-rich 1 (GRXCR1), cause non-syndromic sensorineural deafness. To understand the deglutathionylating enzyme function of GRXCR1 in deafness, we generated two grxcr1 zebrafis...

from #Head and Neck by Sfakianakis via simeraentaxei on Inoreader https://ift.tt/2P3cbhN