Published in Hear Res on February 01, 2004
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Human mitochondrial transcription factor B1 as a modifier gene for hearing loss associated with the mitochondrial A1555G mutation. Mol Genet Metab (2004) 1.81
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The R109H variant of fascin-2, a developmentally regulated actin crosslinker in hair-cell stereocilia, underlies early-onset hearing loss of DBA/2J mice. J Neurosci (2010) 1.54
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Ahl2, a second locus affecting age-related hearing loss in mice. Genomics (2002) 1.35
A missense mutation in the previously undescribed gene Tmhs underlies deafness in hurry-scurry (hscy) mice. Proc Natl Acad Sci U S A (2005) 1.34
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Gravity receptor function in mice with graded otoconial deficiencies. Hear Res (2004) 1.29
Inheritance patterns of progressive hearing loss in laboratory strains of mice. Brain Res (2009) 1.26
The chloride intracellular channel protein CLIC5 is expressed at high levels in hair cell stereocilia and is essential for normal inner ear function. J Neurosci (2006) 1.24
Biochemical characterization of the mitochondrial tRNASer(UCN) T7511C mutation associated with nonsyndromic deafness. Nucleic Acids Res (2004) 1.24
Mutation of the novel gene Tmie results in sensory cell defects in the inner ear of spinner, a mouse model of human hearing loss DFNB6. Hum Mol Genet (2002) 1.24
Genetic background effects on age-related hearing loss associated with Cdh23 variants in mice. Hear Res (2011) 1.23
A locus on distal chromosome 11 (ahl8) and its interaction with Cdh23 ahl underlie the early onset, age-related hearing loss of DBA/2J mice. Genomics (2008) 1.12
Cu/Zn superoxide dismutase and age-related hearing loss. Hear Res (2005) 1.11
Acoustic startle and prepulse inhibition in 40 inbred strains of mice. Behav Neurosci (2003) 1.11
New intragenic deletions in the Phex gene clarify X-linked hypophosphatemia-related abnormalities in mice. Mamm Genome (2004) 1.10
A locus on distal chromosome 10 (ahl4) affecting age-related hearing loss in A/J mice. Neurobiol Aging (2008) 1.10
A missense mutation in the mouse Col2a1 gene causes spondyloepiphyseal dysplasia congenita, hearing loss, and retinoschisis. J Bone Miner Res (2003) 1.09
A quantitative survey of gravity receptor function in mutant mouse strains. J Assoc Res Otolaryngol (2005) 1.08
Pus3p- and Pus1p-dependent pseudouridylation of steroid receptor RNA activator controls a functional switch that regulates nuclear receptor signaling. Mol Endocrinol (2006) 1.06
The podosomal-adaptor protein SH3PXD2B is essential for normal postnatal development. Mamm Genome (2009) 1.05
A comparison of vestibular and auditory phenotypes in inbred mouse strains. Brain Res (2006) 1.03
Dietary thyroid hormone replacement ameliorates hearing deficits in hypothyroid mice. Mamm Genome (2007) 1.02
A missense mutation in the conserved C2B domain of otoferlin causes deafness in a new mouse model of DFNB9. Hear Res (2007) 1.02
Mouse H6 Homeobox 1 (Hmx1) mutations cause cranial abnormalities and reduced body mass. BMC Dev Biol (2009) 1.02
Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3. Mol Genet Metab (2004) 1.01
Mitochondrial ribosomal proteins: candidate genes for mitochondrial disease. Genet Med (2004) 1.00
β-Actin and fascin-2 cooperate to maintain stereocilia length. J Neurosci (2013) 0.99
A novel spontaneous mutation of Irs1 in mice results in hyperinsulinemia, reduced growth, low bone mass and impaired adipogenesis. J Endocrinol (2009) 0.97
Gene responsible for mitochondrial myopathy and sideroblastic anemia (MSA) maps to chromosome 12q24.33. Am J Med Genet A (2004) 0.97
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Human TRMU encoding the mitochondrial 5-methylaminomethyl-2-thiouridylate-methyltransferase is a putative nuclear modifier gene for the phenotypic expression of the deafness-associated 12S rRNA mutations. Biochem Biophys Res Commun (2006) 0.92
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A mouse model with postnatal endolymphatic hydrops and hearing loss. Hear Res (2008) 0.89
Pleiotropic effects and compensation mechanisms determine tissue specificity in mitochondrial myopathy and sideroblastic anemia (MLASA). Mol Genet Metab (2007) 0.87
Targeted knockout and lacZ reporter expression of the mouse Tmhs deafness gene and characterization of the hscy-2J mutation. Mamm Genome (2007) 0.87
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CLIC5 stabilizes membrane-actin filament linkages at the base of hair cell stereocilia in a molecular complex with radixin, taperin, and myosin VI. Cytoskeleton (Hoboken) (2013) 0.83
Otitis media in a new mouse model for CHARGE syndrome with a deletion in the Chd7 gene. PLoS One (2012) 0.83
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A modifier gene alleviates hypothyroidism-induced hearing impairment in Pou1f1dw dwarf mice. Genetics (2011) 0.81
Cochlear developmental defect and background-dependent hearing thresholds in the Jackson circler (jc) mutant mouse. Hear Res (2006) 0.81
A mutation in the cdh23 gene causes age-related hearing loss in Cdh23(nmf308/nmf308) mice. Gene (2012) 0.81
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MRPS18CP2 alleles and DEFA3 absence as putative chromosome 8p23.1 modifiers of hearing loss due to mtDNA mutation A1555G in the 12S rRNA gene. BMC Med Genet (2007) 0.79
A new mutation of the Atoh1 gene in mice with normal life span allows analysis of inner ear and cerebellar phenotype in aging. PLoS One (2013) 0.79
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Phenotypic expression of maternally inherited deafness is affected by RNA modification and cytoplasmic ribosomal proteins. Mol Genet Metab (2009) 0.78
Assessment criteria for rotated stereociliary bundles in the guinea pig cochlea. Otol Neurotol (2008) 0.77
Mutation in Phex gene predisposes BALB/c-Phex(Hyp-Duk)/Y mice to otitis media. PLoS One (2012) 0.77
A mouse model validates the utility of electrocochleography in verifying endolymphatic hydrops. J Assoc Res Otolaryngol (2014) 0.77
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Fine Mapping of a Deafness Mutation hml on Mouse Chromosome 10. Acad J Xian Jiaotong Univ (2004) 0.75
Small papillary tumor in the saccule. Int J Pediatr Otorhinolaryngol Extra (2007) 0.75
Retrotransposon insertion in the T-cell acute lymphocytic leukemia 1 (Tal1) gene is associated with severe renal disease and patchy alopecia in Hairpatches (Hpt) mice. PLoS One (2013) 0.75
Foreword for special issue: mouse models for hearing research. Brain Res (2009) 0.75
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