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

Blanco-Sánchez, B., Clément, A., Fierro, J., Stednitz, S., Phillips, J., Wegner, J., et al. (2018). Grxcr1 Promotes Hair Bundle Development by Destabilizing the Physical Interaction between Harmonin and Sans Usher Syndrome Proteins. Cell Reports, 25(5), 1281-1291. doi:10.1016/j.celrep.2018.10.005.

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Blanco-Sánchez , B, Author
Clément , A, Author
Fierro, J, Author
Stednitz, S1, Author           
Phillips, JB, Author
Wegner, J, Author
Panlilio, JM, Author
Peirce, JL, Author
Washbourne , P, Author
Westerfield, M, Author
Affiliations:
1External Organizations, ou_persistent22              

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 Abstract: Morphogenesis 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 zebrafish mutant alleles. We found that hair bundles are thinner in homozygous grxcr1 mutants, similar to the USH1 mutants ush1c (Harmonin) and ush1ga (Sans). In vitro assays showed that glutathionylation promotes the interaction between Ush1c and Ush1ga and that Grxcr1 regulates mechanoreceptor development by preventing physical interaction between these proteins without affecting the assembly of another USH1 protein complex, the Ush1c-Cadherin23-Myosin7aa tripartite complex. By elucidating the molecular mechanism through which Grxcr1 functions, we also identify a mechanism that dynamically regulates the formation of Usher protein complexes.

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 Dates: 2018-10
 Publication Status: Issued
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 Rev. Type: -
 Identifiers: DOI: 10.1016/j.celrep.2018.10.005
eDoc: e5
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Title: Cell Reports
Source Genre: Journal
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Publ. Info: Maryland Heights, MO : Cell Press
Pages: - Volume / Issue: 25 (5) Sequence Number: - Start / End Page: 1281 - 1291 Identifier: ISSN: 2211-1247
CoNE: https://pure.mpg.de/cone/journals/resource/2211-1247