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TMIE Is an Essential Component of the Mechanotransduction Machinery of Cochlear Hair Cells
TMHS Is an Integral Component of the Mechanotransduction Machinery of Cochlear Hair Cells
Harmonin Mutations Cause Mechanotransduction Defects in Cochlear Hair Cells
Subunit determination of the conductance of hair-cell mechanotransducer channels
- M. Beurg, Wei Xiong, Bo Zhao, U. Müller, R. Fettiplace
- BiologyProceedings of the National Academy of Sciences
- 30 December 2014
The unitary conductance of outer hair cell mechanotransducer (MT) channels was reduced relative to wild type, and the tonotopic gradient in conductance, where channels from the cochlear base are nearly twice as conducting as those at the apex, was almost absent.
A mouse model for nonsyndromic deafness (DFNB12) links hearing loss to defects in tip links of mechanosensory hair cells
- Martin Schwander, Wei Xiong, U. Müller
- Biology, MedicineProceedings of the National Academy of Sciences
- 31 March 2009
The findings suggest that DFNB12 belongs to a new class of disorder that is caused by defects in tip links, and it is proposed that mutations in other genes that cause USH1 and nonsyndromic deafness may also have distinct effects on hair cell development and function.
Structure of the human voltage-gated sodium channel Nav1.4 in complex with beta1
Cryo-electron microscopy structure of the human Nav1.4-β1 complex provides insight into the molecular basis for Na+ permeation and kinetic asymmetry of the four repeats, and corroborates an allosteric blocking mechanism for fast inactivation of Nav channels.
Structure of the human voltage-gated sodium channel Nav1.4 in complex with β1
The high-resolution structure of a human voltage-gated sodium channel, and the structures of an insect Nav channel bound to the toxins that cause pufferfish and shellfish poisoning in humans, give insight into the molecular basis of sodium ion permeation and provide a path toward structure-based drug discovery.
Improved calcium sensor GCaMP-X overcomes the calcium channel perturbations induced by the calmodulin in GCaMP
The intrinsic problem prevailing over different versions and applications is unveiled, showing that GCaMP containing CaM (calmodulin) interferes with both gating and signaling of L-type calcium channels (CaV1) and the development ofGCaMP-X is developed to overcome these limitations.
Regulation of PCDH15 function in mechanosensory hair cells by alternative splicing of the cytoplasmic domain
These findings reveal an essential role for PCDH15-CD2 in the formation of kinociliary links and hair bundle polarization, and show that several PC DH15 isoforms can function redundantly at tip links.
Using injectoporation to deliver genes to mechanosensory hair cells
A method termed injectoporation is described that combines tissue microinjection with electroporation to express cDNAs and shRNAs in mouse cochlear hair cells and it is compatible with the analysis of hair cell function using imaging approaches and electrophysiology.