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Article

Complexes of Usher proteins preassemble at the endoplasmic reticulum and are required for trafficking and ER homeostasis.

Disease models & mechanisms - 1 May 2014

Blanco-Sánchez Bernardo, Clément Aurélie, Fierro Javier, Washbourne Philip, Westerfield Monte

Abstract excerpt

Usher syndrome (USH), the leading cause of hereditary combined hearing and vision loss, is characterized by sensorineural deafness and progressive retinal degeneration. Mutations in several different genes produce USH, but the proximal cause of sensory cell death remains mysterious. We adapted a proximity ligation assay to analyze associations among three of the USH proteins, Cdh23, Harmonin and Myo7aa, and the...

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Identifiers

PMID: 24626987
DOI: 10.1242/dmm.014068
PMCID: PMC4007406

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Complexes of Usher proteins preassemble at the endoplasmic reticulum and are required for trafficking and ER homeostasis.PMID 24626987Read ↗

Characterization of the ternary Usher syndrome SANS/ush2a/whirlin protein complex.Semantic proximity Read ↗

Molecular basis of human Usher syndrome: deciphering the meshes of the Usher protein network provides insights into the pathomechanisms of the Usher disease.Semantic proximity Read ↗

Localization of PDZD7 to the Stereocilia Ankle-Link Associates this Scaffolding Protein with the Usher Syndrome Protein NetworkSemantic proximity Read ↗

Usher syndrome: animal models, retinal function of Usher proteins, and prospects for gene therapy.Semantic proximity Read ↗

Usher protein functions in hair cells and photoreceptorsSemantic proximity Read ↗

Usher proteins in inner ear structure and function.Semantic proximity Read ↗

Molecular analysis of the supramolecular usher protein complex in the retina. Harmonin as the key protein of the Usher syndrome.Semantic proximity Read ↗

Expression and subcellular localization of <i>USH1C</i> /harmonin in the human retina provide insights into pathomechanisms and therapySemantic proximity Read ↗

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Usherin, the defective protein in Usher syndrome type IIA, is likely to be a component of interstereocilia ankle links in the inner ear sensory cellsSemantic proximity Read ↗

Zebrafish Models for the Mechanosensory Hair Cell Dysfunction in Usher Syndrome 3 Reveal That Clarin-1 Is an Essential Hair Bundle Protein.Semantic proximity Read ↗

Homozygous disruption of PDZD7 by reciprocal translocation in a consanguineous family: a new member of the Usher syndrome protein interactome causing congenital hearing impairmentSemantic proximity Read ↗

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