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https://hdl.handle.net/2440/79653
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dc.contributor.author | Molina, L. | - |
dc.contributor.author | Fasquelle, L. | - |
dc.contributor.author | Nouvian, R. | - |
dc.contributor.author | Salvetat, N. | - |
dc.contributor.author | Scott, H. | - |
dc.contributor.author | Guipponi, M. | - |
dc.contributor.author | Molina, F. | - |
dc.contributor.author | Puel, J. | - |
dc.contributor.author | Delprat, B. | - |
dc.date.issued | 2013 | - |
dc.identifier.citation | Human Molecular Genetics, 2013; 22(7):1289-1299 | - |
dc.identifier.issn | 0964-6906 | - |
dc.identifier.issn | 1460-2083 | - |
dc.identifier.uri | http://hdl.handle.net/2440/79653 | - |
dc.description.abstract | Before acquiring their mature state, cochlear hair cells undergo a series of changes in expression of ion channels. How this complex mechanism is achieved is not fully understood. Tmprss3, a type II serine protease expressed in hair cells, is required for their proper functioning at the onset of hearing. To unravel the role of Tmprss3 in the acquisition of mature K+ currents, we compared their function by patch-clamp technique in wild-type Tmprss3WT and Tmprss3Y260X-mutant mice. Interestingly, only outward K+ currents were altered in Tmprss3Y260X-mutant mice. To determine by which mechanism this occurred, we compared the protein network of Tmprss3WT and Tmprss3Y260X-mutant mice using proteomic analysis. This led to the identification of a pathway related to potassium Kcnma1 channels. This pathway was validated by immunohistochemistry, focusing on the most downregulated protein that was identified as a cochlear Kcnma1-associated protein, APOA1. Finally, we show that, in contrast to Tmprss3WT, Kcnma1 channels were absent at the neck of inner hair cells (IHCs) in Tmprss3Y260X-mutant mice. In conclusion, our data suggest that lack of Tmprss3 leads to a decrease in Kcnma1 potassium channels expression in (IHCs). | - |
dc.description.statementofresponsibility | Laurence Molina, Lydie Fasquelle, Régis Nouvian, Nicolas Salvetat, Hamish S. Scott, Michel Guipponi, Franck Molina, Jean-Luc Puel and Benjamin Delprat | - |
dc.language.iso | en | - |
dc.publisher | Oxford Univ Press | - |
dc.rights | © The Author 2012. Published by Oxford University Press. All rights reserved. | - |
dc.source.uri | http://dx.doi.org/10.1093/hmg/dds532 | - |
dc.subject | Cochlea | - |
dc.subject | Animals | - |
dc.subject | Mice, Transgenic | - |
dc.subject | Humans | - |
dc.subject | Mice | - |
dc.subject | Potassium | - |
dc.subject | Apolipoprotein A-I | - |
dc.subject | Membrane Proteins | - |
dc.subject | Proteome | - |
dc.subject | Electrophoresis, Gel, Two-Dimensional | - |
dc.subject | Patch-Clamp Techniques | - |
dc.subject | Gene Expression | - |
dc.subject | Down-Regulation | - |
dc.subject | Membrane Potentials | - |
dc.subject | Protein Transport | - |
dc.subject | Mutation, Missense | - |
dc.subject | Large-Conductance Calcium-Activated Potassium Channel alpha Subunits | - |
dc.subject | Tandem Mass Spectrometry | - |
dc.subject | Metabolic Networks and Pathways | - |
dc.subject | Hair Cells, Auditory, Inner | - |
dc.subject | Serine Proteases | - |
dc.title | Tmprss3 loss of function impairs cochlear inner hair cell Kcnma1 channel membrane expression | - |
dc.type | Journal article | - |
dc.identifier.doi | 10.1093/hmg/dds532 | - |
pubs.publication-status | Published | - |
dc.identifier.orcid | Scott, H. [0000-0002-5813-631X] | - |
Appears in Collections: | Aurora harvest Molecular and Biomedical Science publications |
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