Structures of Usher Syndrome 1 Proteins and Their Complexes

How the Genetics of Deafness Illuminates Auditory Physiology

(2011) Guy P. Richardson et al.   Annual Review of Physiology

Regulation of PCDH15 function in mechanosensory hair cells by alternative splicing of the cytoplasmic domain

(2011) S. W. Webb et al.   DEVELOPMENT

Structural basis of cargo recognition by the myosin-X MyTH4-FERM domain

(2011) Yoshinori Hirano et al.   EMBO JOURNAL

The Kinetic Mechanism of Mouse Myosin VIIA

(2011) Jessica Haithcock et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Characterization of a Myosin VII MyTH/FERM Domain

(2011) Rebecca J. Moen et al.   JOURNAL OF MOLECULAR BIOLOGY

Forces between clustered stereocilia minimize friction in the ear on a subnanometre scale

(2011) Andrei S. Kozlov et al.   NATURE

Harmonin inhibits presynaptic Cav1.3 Ca2+ channels in mouse inner hair cells

(2011) Frederick D Gregory et al.   NATURE NEUROSCIENCE

Myosin VIIa and sans localization at stereocilia upper tip-link density implicates these Usher syndrome proteins in mechanotransduction

(2011) M. Grati et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cargo recognition mechanism of myosin X revealed by the structure of its tail MyTH4-FERM tandem in complex with the DCC P3 domain

(2011) Zhiyi Wei et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cargo binding activates myosin VIIA motor function in cells

(2011) T. Sakai et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Usher type 1G protein sans is a critical component of the tip-link complex, a structure controlling actin polymerization in stereocilia

(2011) E. Caberlotto et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Connecting the ear to the brain: Molecular mechanisms of auditory circuit assembly

(2011) Jessica M. Appler et al.   PROGRESS IN NEUROBIOLOGY

Structure of MyTH4-FERM Domains in Myosin VIIa Tail Bound to Cargo

(2011) L. Wu et al.   SCIENCE

Actin-Bundling Protein TRIOBP Forms Resilient Rootlets of Hair Cell Stereocilia Essential for Hearing

(2010) Shin-ichiro Kitajiri et al.   CELL

The prevalence of Usher syndrome and other retinal dystrophy-hearing impairment associations

(2010) Thomas Rosenberg et al.   CLINICAL GENETICS

Cadherin-23, myosin VIIa and harmonin, encoded by Usher syndrome type I genes, form a ternary complex and interact with membrane phospholipids

(2010) Amel Bahloul et al.   HUMAN MOLECULAR GENETICS

Analysis of subcellular localization of Myo7a, Pcdh15 and Sans in Ush1c knockout mice

(2010) Denise Yan et al.   INTERNATIONAL JOURNAL OF EXPERIMENTAL PATHOLOGY

Asymmetric distribution of cadherin 23 and protocadherin 15 in the kinocilial links of avian sensory hair cells

(2010) Richard J. Goodyear et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Genetics and pathological mechanisms of Usher syndrome

(2010) Denise Yan et al.   JOURNAL OF HUMAN GENETICS

Targeting of the Hair Cell Proteins Cadherin 23, Harmonin, Myosin XVa, Espin, and Prestin in an Epithelial Cell Model

(2010) L. Zheng et al.   JOURNAL OF NEUROSCIENCE

Structural Determinants of Cadherin-23 Function in Hearing and Deafness

(2010) Marcos Sotomayor et al.   NEURON

The structure of the harmonin/sans complex reveals an unexpected interaction mode of the two Usher syndrome proteins

(2010) J. Yan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structure of the N terminus of cadherin 23 reveals a new adhesion mechanism for a subset of cadherin superfamily members

(2010) H. M. Elledge et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An Update on the Genetics of Usher Syndrome

(2010) José M. Millán et al.   Journal of Ophthalmology

Mechanotransduction by Hair Cells: Models, Molecules, and Mechanisms

(2009) Peter G. Gillespie et al.   CELL

Myosin VI Undergoes Cargo-Mediated Dimerization

(2009) Cong Yu et al.   CELL

Function and Expression Pattern of Nonsyndromic Deafness Genes

(2009) Nele Hilgert et al.   CURRENT MOLECULAR MEDICINE

Linking genes underlying deafness to hair-bundle development and function

(2009) Christine Petit et al.   NATURE NEUROSCIENCE

Localization of inner hair cell mechanotransducer channels using high-speed calcium imaging

(2009) Maryline Beurg et al.   NATURE NEUROSCIENCE

Organization and dynamics of PDZ-domain-related supramodules in the postsynaptic density

(2009) Wei Feng et al.   NATURE REVIEWS NEUROSCIENCE

Harmonin Mutations Cause Mechanotransduction Defects in Cochlear Hair Cells

(2009) Nicolas Grillet et al.   NEURON

The tail binds to the head-neck domain, inhibiting ATPase activity of myosin VIIA

(2009) N. Umeki et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A FERM domain autoregulates Drosophila myosin 7a activity

(2009) Y. Yang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Assembling stable hair cell tip link complex via multidentate interactions between harmonin and cadherin 23

(2009) L. Pan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structure and Biochemistry of Cadherins and Catenins

(2009) L. Shapiro et al.   Cold Spring Harbor Perspectives in Biology

Cadherins and mechanotransduction by hair cells

(2008) Ulrich Müller   CURRENT OPINION IN CELL BIOLOGY

A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growth

(2008) G. Lefevre et al.   DEVELOPMENT

Human hereditary hearing impairment: mouse models can help to solve the puzzle

(2008) Karen Vrijens et al.   HUMAN GENETICS

Three-dimensional Architecture of Hair-bundle Linkages Revealed by Electron-microscopic Tomography

(2008) Manfred Auer et al.   JARO-JOURNAL OF THE ASSOCIATION FOR RESEARCH IN OTOLARYNGOLOGY

MAGI-1, A Candidate Stereociliary Scaffolding Protein, Associates with the Tip-Link Component Cadherin 23

(2008) Z. Xu et al.   JOURNAL OF NEUROSCIENCE

The Dimensions and Composition of Stereociliary Rootlets in Mammalian Cochlear Hair Cells: Comparison between High- and Low-Frequency Cells and Evidence for a Connection to the Lateral Membrane

(2008) D. N. Furness et al.   JOURNAL OF NEUROSCIENCE

Stereocilin-deficient mice reveal the origin of cochlear waveform distortions

(2008) Elisabeth Verpy et al.   NATURE

Making an Effort to Listen: Mechanical Amplification in the Ear

(2008) A.J. Hudspeth   NEURON

Usher syndrome: Animal models, retinal function of Usher proteins, and prospects for gene therapy

(2007) David S. Williams   VISION RESEARCH