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Cadherin 23 and protocadherin 15 interact to form tip-link filaments in sensory hair cells
TLDR
The studies define the molecular composition of tip links and provide a conceptual base for exploring the mechanisms of sensory impairment associated with mutations in CDH23 and PCDH15. Expand
β1-Class Integrins Regulate the Development of Laminae and Folia in the Cerebral and Cerebellar Cortex
TLDR
The phenotype of the beta1-deficient mice resembles pathological changes observed in human cortical dysplasias, suggesting that defective integrin-mediated signal transduction contributes to the development of some of these diseases. Expand
Cadherin 23 is a component of the tip link in hair-cell stereocilia
TLDR
It is shown that antibodies against CDH23 label the tip link, and that cadherin 23 has biochemical properties similar to those of theTip link, suggesting thatCDH23 and myosin-1c cooperate to regulate the activity of mechanically gated ion channels in hair cells. Expand
Fate-Restricted Neural Progenitors in the Mammalian Cerebral Cortex
TLDR
It is demonstrated that the murine cerebral cortex contains RGC sublineages with distinct fate potentials, and an RGC lineage is identified that is intrinsically specified to generate only upper-layer neurons, independently of niche and birthdate. Expand
Reelin Regulates Cadherin Function via Dab1/Rap1 to Control Neuronal Migration and Lamination in the Neocortex
TLDR
The cellular mechanism of reelin function during radial migration is defined, the molecular pathway downstream of Dab1 during somal translocation is elucidated, and the importance of glia-independent motility in neocortical development is established. Expand
The Usher syndrome proteins cadherin 23 and harmonin form a complex by means of PDZ-domain interactions
TLDR
It is shown here that CDH23 and harmonin form a protein complex that is predicted to disrupt stereocilia bundles and cause deafness in USH1 patients. Expand
Mechanotransduction by Hair Cells: Models, Molecules, and Mechanisms
TLDR
The recent discovery of several molecular constituents of the mechanotransduction machinery of hair cells provides a new framework for the interpretation of biophysical data and necessitates revision of prevailing models of mechanotranduction. Expand
Beta1 integrins regulate myoblast fusion and sarcomere assembly.
TLDR
It is shown that extracellular matrix (ECM) receptors of the beta1 integrin family regulate myoblast fusion, providing evidence that different ECM receptors have nonredundant functions in skeletal muscle fibers. Expand
TMHS Is an Integral Component of the Mechanotransduction Machinery of Cochlear Hair Cells
TLDR
It is shown that mechanotransduction is impaired in mice lacking the tetraspan TMHS, which resembles other ion channel regulatory subunits such as the transmembrane alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor regulatory proteins (TARPs) of AMPA receptors that facilitate channel transport and regulate the properties of pore-forming channel subunits. Expand
Hearing requires otoferlin-dependent efficient replenishment of synaptic vesicles in hair cells
TLDR
It is concluded that replenishment does not match the release of vesicles at mutant active zones in vivo and a sufficient standing RRP therefore cannot be maintained and it is proposed that otoferlin is involved in replenishing synaptic vesicle replenishment. Expand
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