Published in Biochemistry on February 16, 2010
Role for a novel Usher protein complex in hair cell synaptic maturation. PLoS One (2012) 0.98
Usher protein functions in hair cells and photoreceptors. Int J Biochem Cell Biol (2013) 0.91
Regulated vesicular trafficking of specific PCDH15 and VLGR1 variants in auditory hair cells. J Neurosci (2012) 0.85
Current understanding of usher syndrome type II. Front Biosci (Landmark Ed) (2012) 0.85
Clarin-1 acts as a modulator of mechanotransduction activity and presynaptic ribbon assembly. J Cell Biol (2014) 0.79
The mitotic spindle protein SPAG5/Astrin connects to the Usher protein network postmitotically. Cilia (2012) 0.79
Accelerated age-related olfactory decline among type 1 Usher patients. Sci Rep (2016) 0.75
Defective myosin VIIA gene responsible for Usher syndrome type 1B. Nature (1995) 5.91
Cadherin 23 and protocadherin 15 interact to form tip-link filaments in sensory hair cells. Nature (2007) 4.87
Rhodopsin content in the outer segment membranes of bovine and frog retinal rods. Biochemistry (1974) 4.34
A defect in harmonin, a PDZ domain-containing protein expressed in the inner ear sensory hair cells, underlies Usher syndrome type 1C. Nat Genet (2000) 3.71
Cadherin 23 is a component of the tip link in hair-cell stereocilia. Nature (2004) 3.69
Myosin VIIa, harmonin and cadherin 23, three Usher I gene products that cooperate to shape the sensory hair cell bundle. EMBO J (2002) 3.53
Usher syndrome 1D and nonsyndromic autosomal recessive deafness DFNB12 are caused by allelic mutations of the novel cadherin-like gene CDH23. Am J Hum Genet (2000) 3.48
Mutations of the protocadherin gene PCDH15 cause Usher syndrome type 1F. Am J Hum Genet (2001) 3.42
Mutation of CDH23, encoding a new member of the cadherin gene family, causes Usher syndrome type 1D. Nat Genet (2001) 3.15
The fragile X syndrome protein represses activity-dependent translation through CYFIP1, a new 4E-BP. Cell (2008) 3.10
Usher syndrome: definition and estimate of prevalence from two high-risk populations. J Chronic Dis (1983) 2.98
A recessive contiguous gene deletion causing infantile hyperinsulinism, enteropathy and deafness identifies the Usher type 1C gene. Nat Genet (2000) 2.87
Mutation of a gene encoding a protein with extracellular matrix motifs in Usher syndrome type IIa. Science (1998) 2.84
Mutations in the novel protocadherin PCDH15 cause Usher syndrome type 1F. Hum Mol Genet (2001) 2.48
Usherin, the defective protein in Usher syndrome type IIA, is likely to be a component of interstereocilia ankle links in the inner ear sensory cells. Hum Mol Genet (2005) 2.38
Usher syndrome type I G (USH1G) is caused by mutations in the gene encoding SANS, a protein that associates with the USH1C protein, harmonin. Hum Mol Genet (2003) 2.37
The Usher syndrome proteins cadherin 23 and harmonin form a complex by means of PDZ-domain interactions. Proc Natl Acad Sci U S A (2002) 2.34
Usher syndrome: molecular links of pathogenesis, proteins and pathways. Hum Mol Genet (2006) 2.30
A novel gene for Usher syndrome type 2: mutations in the long isoform of whirlin are associated with retinitis pigmentosa and sensorineural hearing loss. Hum Genet (2006) 2.27
Molecular basis of human Usher syndrome: deciphering the meshes of the Usher protein network provides insights into the pathomechanisms of the Usher disease. Exp Eye Res (2006) 2.19
Interactions in the network of Usher syndrome type 1 proteins. Hum Mol Genet (2004) 2.16
A novel Usher protein network at the periciliary reloading point between molecular transport machineries in vertebrate photoreceptor cells. Hum Mol Genet (2007) 2.11
Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly. Hum Mol Genet (2004) 2.04
The DFNB31 gene product whirlin connects to the Usher protein network in the cochlea and retina by direct association with USH2A and VLGR1. Hum Mol Genet (2006) 1.94
The very large G-protein-coupled receptor VLGR1: a component of the ankle link complex required for the normal development of auditory hair bundles. J Neurosci (2006) 1.93
Retinitis pigmentosa combined with congenital deafness; with vestibulo-cerebellar ataxia and mental abnormality in a proportion of cases: A clinical and genetico-statistical study. Acta Psychiatr Scand Suppl (1959) 1.89
Identification of 51 novel exons of the Usher syndrome type 2A (USH2A) gene that encode multiple conserved functional domains and that are mutated in patients with Usher syndrome type II. Am J Hum Genet (2004) 1.88
Usher syndrome: from genetics to pathogenesis. Annu Rev Genomics Hum Genet (2001) 1.87
Usher I syndrome: unravelling the mechanisms that underlie the cohesion of the growing hair bundle in inner ear sensory cells. J Cell Sci (2005) 1.84
Mouse models of USH1C and DFNB18: phenotypic and molecular analyses of two new spontaneous mutations of the Ush1c gene. Hum Mol Genet (2003) 1.83
Spatiotemporal pattern and isoforms of cadherin 23 in wild type and waltzer mice during inner ear hair cell development. Dev Biol (2005) 1.82
Sequential pulses of apical epithelial secretion and endocytosis drive airway maturation in Drosophila. Dev Cell (2007) 1.79
Physical and functional interaction between protocadherin 15 and myosin VIIa in mechanosensory hair cells. J Neurosci (2006) 1.75
The molecular genetics of Usher syndrome. Clin Genet (2003) 1.68
Endoplasmic reticulum stress is implicated in retinal inflammation and diabetic retinopathy. FEBS Lett (2009) 1.65
Harmonin mutations cause mechanotransduction defects in cochlear hair cells. Neuron (2009) 1.63
Polarized sorting of rhodopsin on post-Golgi membranes in frog retinal photoreceptor cells. J Cell Biol (1991) 1.63
Dystrophia retinae pigmentosa--dysacusis syndrome (DRD): a study of the Usher- or Hallgren syndrome. J Genet Hum (1970) 1.55
Scaffold protein harmonin (USH1C) provides molecular links between Usher syndrome type 1 and type 2. Hum Mol Genet (2005) 1.54
Assignment of an Usher syndrome type III (USH3) gene to chromosome 3q. Hum Mol Genet (1995) 1.42
Myosin VIIa as a common component of cilia and microvilli. Cell Motil Cytoskeleton (1998) 1.37
USH3A transcripts encode clarin-1, a four-transmembrane-domain protein with a possible role in sensory synapses. Eur J Hum Genet (2002) 1.36
Neurodegeneration of mouse nigrostriatal dopaminergic system induced by repeated oral administration of rotenone is prevented by 4-phenylbutyrate, a chemical chaperone. J Neurochem (2007) 1.34
Localization and expression of clarin-1, the Clrn1 gene product, in auditory hair cells and photoreceptors. Hear Res (2009) 1.26
Photoreceptor expression of the Usher syndrome type 1 protein protocadherin 15 (USH1F) and its interaction with the scaffold protein harmonin (USH1C). Mol Vis (2005) 1.25
Cadherins and mechanotransduction by hair cells. Curr Opin Cell Biol (2008) 1.23
Very large G protein-coupled receptor-1, the largest known cell surface protein, is highly expressed in the developing central nervous system. J Biol Chem (2001) 1.22
Differential distribution of harmonin isoforms and their possible role in Usher-1 protein complexes in mammalian photoreceptor cells. Invest Ophthalmol Vis Sci (2003) 1.13
A novel D458V mutation in the SANS PDZ binding motif causes atypical Usher syndrome. J Mol Med (Berl) (2005) 1.07
Gene structure and mutant alleles of PCDH15: nonsyndromic deafness DFNB23 and type 1 Usher syndrome. Hum Genet (2008) 1.07
Localization and expression of usherin: a novel basement membrane protein defective in people with Usher's syndrome type IIa. Hear Res (2002) 1.06
The unconventional myosin-VIIa associates with lysosomes. Cell Motil Cytoskeleton (2005) 1.03
Polypeptide chains of 19-S thyroglobulin from several mammalian species and of porcine 27-S iodoprotein. Eur J Biochem (1977) 1.01
A domain-specific usherin/collagen IV interaction may be required for stable integration into the basement membrane superstructure. J Cell Sci (2004) 0.98
Molecular analysis of the supramolecular usher protein complex in the retina. Harmonin as the key protein of the Usher syndrome. Adv Exp Med Biol (2006) 0.95
SANS (USH1G) expression in developing and mature mammalian retina. Vision Res (2007) 0.93
Usherin expression is highly conserved in mouse and human tissues. Hear Res (2002) 0.93
Unconventional myosins, the basis for deafness in mouse and man. Am J Hum Genet (1997) 0.91
Evidence for functional importance of usherin/fibronectin interactions in retinal basement membranes. Biochemistry (2005) 0.84
Physicochemical characterization of ribosomal particles from the eukaryote Artemia. J Biol Chem (1981) 0.83
Roles and interactions of usher 1 proteins in the outer retina. Adv Exp Med Biol (2006) 0.82
Neutralization of circulating vascular endothelial growth factor (VEGF) by anti-VEGF antibodies and soluble VEGF receptor 1 (sFlt-1) induces proteinuria. J Biol Chem (2003) 3.36
Bone-marrow-derived stem cells repair basement membrane collagen defects and reverse genetic kidney disease. Proc Natl Acad Sci U S A (2006) 3.03
Determinants of vascular permeability in the kidney glomerulus. J Biol Chem (2002) 1.93
Human alpha1 type IV collagen NC1 domain exhibits distinct antiangiogenic activity mediated by alpha1beta1 integrin. J Clin Invest (2005) 1.66
Monocytes may promote myofibroblast accumulation and apoptosis in Alport renal fibrosis. Kidney Int (2003) 1.60
Ethanol stimulates ciliary beating by dual cyclic nucleotide kinase activation in bovine bronchial epithelial cells. Am J Pathol (2003) 1.58
Stage-specific action of matrix metalloproteinases influences progressive hereditary kidney disease. PLoS Med (2006) 1.54
Intranasal organic dust exposure-induced airway adaptation response marked by persistent lung inflammation and pathology in mice. Am J Physiol Lung Cell Mol Physiol (2009) 1.50
Lack of integrin alpha1beta1 leads to severe glomerulosclerosis after glomerular injury. Am J Pathol (2004) 1.33
Effects of cigarette smoke and alcohol on ciliated tracheal epithelium and inflammatory cell recruitment. Am J Respir Cell Mol Biol (2006) 1.31
Both cAMP and cGMP are required for maximal ciliary beat stimulation in a cell-free model of bovine ciliary axonemes. Am J Physiol Lung Cell Mol Physiol (2004) 1.28
Localization and expression of clarin-1, the Clrn1 gene product, in auditory hair cells and photoreceptors. Hear Res (2009) 1.26
Primary ciliary dyskinesia in mice lacking the novel ciliary protein Pcdp1. Mol Cell Biol (2007) 1.26
Toll-like receptor 2 regulates organic dust-induced airway inflammation. Am J Respir Cell Mol Biol (2011) 1.24
Desensitization of PKA-stimulated ciliary beat frequency in an ethanol-fed rat model of cigarette smoke exposure. Alcohol Clin Exp Res (2004) 1.24
Role for macrophage metalloelastase in glomerular basement membrane damage associated with alport syndrome. Am J Pathol (2006) 1.20
Nitric oxide-dependent cilia regulatory enzyme localization in bovine bronchial epithelial cells. J Histochem Cytochem (2007) 1.15
Long-term cigarette smoke exposure in a mouse model of ciliated epithelial cell function. Am J Respir Cell Mol Biol (2009) 1.14
Differential in vivo effects of whole cigarette smoke exposure versus cigarette smoke extract on mouse ciliated tracheal epithelium. Exp Lung Res (2006) 1.11
Stem cell therapy for Alport syndrome: the hope beyond the hype. Nephrol Dial Transplant (2008) 1.07
Localization and expression of usherin: a novel basement membrane protein defective in people with Usher's syndrome type IIa. Hear Res (2002) 1.06
Increased expression of MMP-2, MMP-9 (type IV collagenases/gelatinases), and MT1-MMP in canine X-linked Alport syndrome (XLAS). Kidney Int (2003) 1.05
Hybrid malondialdehyde and acetaldehyde protein adducts form in the lungs of mice exposed to alcohol and cigarette smoke. Alcohol Clin Exp Res (2011) 1.05
Biomechanical strain causes maladaptive gene regulation, contributing to Alport glomerular disease. Kidney Int (2009) 1.03
Direct involvement of the isotype-specific C-terminus of beta tubulin in ciliary beating. J Cell Sci (2005) 0.99
Co-exposure to cigarette smoke and alcohol decreases airway epithelial cell cilia beating in a protein kinase Cε-dependent manner. Am J Pathol (2012) 0.99
Role for a novel Usher protein complex in hair cell synaptic maturation. PLoS One (2012) 0.98
Alcohol increases the permeability of airway epithelial tight junctions in Beas-2B and NHBE cells. Alcohol Clin Exp Res (2011) 0.98
A domain-specific usherin/collagen IV interaction may be required for stable integration into the basement membrane superstructure. J Cell Sci (2004) 0.98
Matrix metalloproteinase dysregulation in the stria vascularis of mice with Alport syndrome: implications for capillary basement membrane pathology. Am J Pathol (2005) 0.98
Malondialdehyde-acetaldehyde-adducted protein inhalation causes lung injury. Alcohol (2011) 0.97
Regulation of COX-2 mediated signaling by alpha3 type IV noncollagenous domain in tumor angiogenesis. Blood (2007) 0.96
α1β1 integrin/Rac1-dependent mesangial invasion of glomerular capillaries in Alport syndrome. Am J Pathol (2013) 0.96
Malondialdehyde-acetaldehyde adducts decrease bronchial epithelial wound repair. Alcohol (2005) 0.95
Alcohol functionally upregulates Toll-like receptor 2 in airway epithelial cells. Alcohol Clin Exp Res (2008) 0.95
Moderate light-induced degeneration of rod photoreceptors with delayed transducin translocation in shaker1 mice. Invest Ophthalmol Vis Sci (2011) 0.93
Inhibition of protein kinase C epsilon causes ciliated bovine bronchial cell detachment. Exp Lung Res (2006) 0.93
Usherin expression is highly conserved in mouse and human tissues. Hear Res (2002) 0.93
Increasing evidence for syndromic phenotypes associated with RPGR mutations. Am J Ophthalmol (2004) 0.92
Ethanol increases phosphodiesterase 4 activity in bovine bronchial epithelial cells. Alcohol (2003) 0.92
Alcohol reduces airway hyperresponsiveness (AHR) and allergic airway inflammation in mice. Am J Physiol Lung Cell Mol Physiol (2011) 0.91
FAK and p38-MAP kinase-dependent activation of apoptosis and caspase-3 in retinal endothelial cells by alpha1(IV)NC1. Invest Ophthalmol Vis Sci (2009) 0.91
Non-typeable Haemophilus influenzae decreases cilia beating via protein kinase Cε. Respir Res (2012) 0.89
RACK1, a PKC targeting protein, is exclusively localized to basal airway epithelial cells. J Histochem Cytochem (2007) 0.89
Loss of SPEF2 function in mice results in spermatogenesis defects and primary ciliary dyskinesia. Biol Reprod (2011) 0.89
Alcohol up-regulates TLR2 through a NO/cGMP dependent pathway. Alcohol Clin Exp Res (2009) 0.88
Proteomic analysis of bovine axonemes exposed to acute alcohol: role of endothelial nitric oxide synthase and heat shock protein 90 in cilia stimulation. Alcohol Clin Exp Res (2012) 0.86
Regulated vesicular trafficking of specific PCDH15 and VLGR1 variants in auditory hair cells. J Neurosci (2012) 0.85
Ethanol blocks adenosine uptake via inhibiting the nucleoside transport system in bronchial epithelial cells. Alcohol Clin Exp Res (2009) 0.85
Progressive morphological and functional defects in retinas from alpha1 integrin-null mice. Invest Ophthalmol Vis Sci (2008) 0.85
Evidence for functional importance of usherin/fibronectin interactions in retinal basement membranes. Biochemistry (2005) 0.84
Smoke extract impairs adenosine wound healing: implications of smoke-generated reactive oxygen species. Am J Respir Cell Mol Biol (2013) 0.83
Dietary antioxidants prevent alcohol-induced ciliary dysfunction. Alcohol (2013) 0.83
Particulate matter in cigarette smoke increases ciliary axoneme beating through mechanical stimulation. J Aerosol Med Pulm Drug Deliv (2012) 0.83
Loss of ASP but not ROPN1 reduces mammalian ciliary motility. Cytoskeleton (Hoboken) (2011) 0.82
Alcohol exposure alters mouse lung inflammation in response to inhaled dust. Nutrients (2012) 0.82
Dysregulation of renal MMP-3 and MMP-7 in canine X-linked Alport syndrome. Pediatr Nephrol (2005) 0.82
Alcohol decreases RhoA activity through a nitric oxide (NO)/cyclic GMP(cGMP)/protein kinase G (PKG)-dependent pathway in the airway epithelium. Alcohol Clin Exp Res (2011) 0.81
Collagen XIII induced in vascular endothelium mediates alpha1beta1 integrin-dependent transmigration of monocytes in renal fibrosis. Am J Pathol (2010) 0.81
Ethanol treatment reduces bovine bronchial epithelial cell migration. Alcohol Clin Exp Res (2005) 0.80
Ethanol attenuates contraction of primary cultured rat airway smooth muscle cells. Am J Respir Cell Mol Biol (2009) 0.80
Deletion of airway cilia results in noninflammatory bronchiectasis and hyperreactive airways. Am J Physiol Lung Cell Mol Physiol (2013) 0.79
Adenosine activation of A(2B) receptor(s) is essential for stimulated epithelial ciliary motility and clearance. Am J Physiol Lung Cell Mol Physiol (2011) 0.79
Protein kinase C epsilon is important in modulating organic-dust-induced airway inflammation. Exp Lung Res (2012) 0.78
Strial marginal cells play a role in basement membrane homeostasis: in vitro and in vivo evidence. Hear Res (2002) 0.78
Motile cilia harbor serum response factor as a mechanism of environment sensing and injury response in the airway. Am J Physiol Lung Cell Mol Physiol (2014) 0.76
Use of a novel cell adhesion method and digital measurement to show stimulus-dependent variation in somatic and oral ciliary beat frequency in Paramecium. J Eukaryot Microbiol (2014) 0.76
Clarin-1 protein expression in photoreceptors. Hear Res (2009) 0.76
Light-induced translocation of RGS9-1 and Gβ5L in mouse rod photoreceptors. PLoS One (2013) 0.76
A complex, tissue-specific role for plasmin and its regulators in modulating fibrogenic activity. Am J Physiol Renal Physiol (2007) 0.75
Transitions experienced by older survivors of critical care. J Gerontol Nurs (2011) 0.75
Immunohistochemistry and reverse transcriptase-polymerase chain reaction as methods for diagnostic determination of usher syndrome type IIa. Laryngoscope (2004) 0.75