Research Topics
Genomes and Genes | Kumar N AlagramamSummaryAffiliation: Case Western Reserve University Country: USA Publications
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Publications
Characterization of vestibular dysfunction in the mouse model for Usher syndrome 1FKumar N Alagramam
Department of Otolaryngology Head and Neck Surgery, University Hospitals of Cleveland, Case Western Reserve University, Cleveland, OH 44106, USA
J Assoc Res Otolaryngol 6:106-18. 2005....- A mouse model with postnatal endolymphatic hydrops and hearing lossCliff A Megerian
Department of Otolaryngology Head and Neck Surgery, Case Western Reserve University, University Hospitals Case Medical Center, 11100 Euclid Avenue, Cleveland, OH 44106, USA
Hear Res 237:90-105. 2008.... - The mechanosensory structure of the hair cell requires clarin-1, a protein encoded by Usher syndrome III causative geneRuishuang Geng
Otolaryngology Head and Neck Surgery, University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, Ohio 44106, USA
J Neurosci 32:9485-98. 2012..Furthermore, the ear phenotype in the Clrn1(N48K) mouse suggests that it is a valuable model for ear disease in CLRN1(N48K), the most prevalent Usher syndrome III mutation in North America... 
Development of outer hair cells in Ames waltzer mice: mutation in protocadherin 15 affects development of cuticular plate and associated structuresYayoi S Kikkawa
Department of Otolaryngology Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, Texas 75390 9035, USA
Anat Rec (Hoboken) 291:224-32. 2008..These observations support the hypothesis that mutations in Pcdh15 in av3J mice adversely affect coordinated maturation of apical cell components, resulting in disturbed stereocilia bundle polarity in av mice...- Molecular changes associated with the endolymphatic hydrops modelSamantha Anne
Otolaryngology Head and Neck Surgery, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio 44106, USA
Otol Neurotol 28:834-41. 2007.... - Identification and characterization of mouse cochlear stem cellsMichael V Yerukhimovich
Department of Otolaryngology, Head and Neck Surgery, Case Western Reserve University, Cleveland, Ohio 44106, USA
Dev Neurosci 29:251-60. 2007..Our results show that cochleae from neonatal mice harbor cells capable of forming spheres and cells from these spheres appear to be better endowed to become hair cells... - Progression of inner ear pathology in Ames waltzer mice and the role of protocadherin 15 in hair cell developmentKaren S Pawlowski
Department of Otolaryngology Head and Neck Surgery, University of Texas Southwestern Medical Center, Dallas, TX 75390 9035, USA
J Assoc Res Otolaryngol 7:83-94. 2006..The positive correlation of severity of effects with extent of mutation can be seen well into adulthood... 
Pharmacological protection of hearing loss in the mouse model of endolymphatic hydropsSami J Melki
Department of Otolaryngology Head and Neck Surgery, University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, Ohio, USA
Laryngoscope 120:1637-45. 2010..This study tests the hypothesis that interruption of the initial and/or downstream aspects of excitotoxicity should provide hearing protection in ELH-associated hearing loss...- Spiral ganglion degeneration patterns in endolymphatic hydropsPaul J Bixenstine
Department of Otolaryngology Head and Neck Surgery, Case Western Reserve University, University Hospitals of Cleveland, Cleveland, Ohio 44106, USA
Laryngoscope 118:1217-23. 2008..The goal of this study is to evaluate the hypothesis that spiral ganglion cell degeneration is the principle pathologic site of ELH-related cochlear injury, correlates with ELH severity, and is most profound in the apical region... - Optimization of ribonucleic acid detection from archival Guinea pig temporal bone specimensKathryn L Hall
Department of Otolaryngology Head and Neck Surgery, University Hospitals of Cleveland, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
Otol Neurotol 28:116-23. 2007..The choice of ribonucleic acid (RNA) isolation protocol coupled with modifications to RNA extraction and detection procedures may result in a more reliable method to detect gene expression in archived temporal bones... - Spiral ganglion loss outpaces inner hair cell loss in endolymphatic hydropsSuhael R Momin
Department of Otolaryngology Head and Neck Surgery, Case Western Reserve University, University Hospitals Case Medical Center, Cleveland, Ohio 44106, USA
Laryngoscope 120:159-65. 2010.... - Usher syndrome IIIA gene clarin-1 is essential for hair cell function and associated neural activationRuishuang Geng
Department of Otolaryngology Head and Neck Surgery, Case Western Reserve University, Cleveland, OH 44106, USA
Hum Mol Genet 18:2748-60. 2009..In sum, hair cell dysfunction and prolonged peak latencies in vestibular and cochlear evoked potentials in Clrn1(-/-) mice strongly indicate that Clrn1 is necessary for hair cell function and associated neural activation... - Mutations in protocadherin 15 and cadherin 23 affect tip links and mechanotransduction in mammalian sensory hair cellsKumar N Alagramam
Otolaryngology Head and Neck Surgery, University Hospitals Case Medical Center, Case Western Reserve University, Cleveland, Ohio, United States of America
PLoS ONE 6:e19183. 2011..These results therefore provide genetic evidence consistent with PCDH15 and CDH23 being part of the tip-link complex and necessary for normal mechanotransduction... - Assessment of retinal structure and function in Ames waltzer miceSherry L Ball
Research Service, Cleveland VA Medical Center, Cleveland, Ohio, USA
Invest Ophthalmol Vis Sci 44:3986-92. 2003..To determine whether the Ames waltzer is a model for retinopathy in USH1F, retinal structure and function were assessed in all four available alleles of the mouse... - Promoter, alternative splice forms, and genomic structure of protocadherin 15Kumar N Alagramam
Department of Otolaryngology Head and Neck Surgery, University Hospitals of Cleveland, Case Western Reserve University, Cleveland, OH 44106, USA
Genomics 90:482-92. 2007..Results from our study show that both mouse and human protocadherin 15 genes have complex genomic structures and transcription control mechanisms... - Tympanometry assessment of 61 inbred strains of miceQing Yin Zheng
Department of Otolaryngology Head and Neck Surgery, Case Western Reserve University, 11100 Euclid Avenue, LKS 5045, Cleveland, OH 44106, USA
Hear Res 231:23-31. 2007..This is the first report to successfully use tympanometry to measure mouse middle ear function, which has been a challenge for the hearing research field because of the mouse's tiny ear size... - Gene expression analysis of distinct populations of cells isolated from mouse and human inner ear FFPE tissue using laser capture microdissection--a technical report based on preliminary findingsNitin A Pagedar
Department of Otolaryngology-Head and Neck Surgery, University Hospitals of Cleveland, Lakeside 4500, 11100 Euclid Avenue, Cleveland, OH 44106, USA
Brain Res 1091:289-99. 2006..Also, this method can be applied for analysis of human archival ear tissue... - The basic science of Meniere's disease and endolymphatic hydropsMaroun T Semaan
Department of Otolaryngology-Head and Neck Surgery, University Hospitals of Cleveland, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
Curr Opin Otolaryngol Head Neck Surg 13:301-7. 2005..SUMMARY: These recent advances have expanded our understanding and will allow for the development of targeted therapeutic interventions aimed at preventing the progression oochleovestibular deterioration... - Interactions in the network of Usher syndrome type 1 proteinsAvital Adato
Unité de Génétique des Déficits Sensoriels, INSERM U587, Institut Pasteur, 25 rue du Dr Roux, 75724 Paris Cedex 15, France
Hum Mol Genet 14:347-56. 2005..We propose that via its binding to myosin VIIa and/or harmonin, sans controls the hair bundle cohesion and proper development by regulating the traffic of USH1 proteins en route to the stereocilia... - A quantitative survey of gravity receptor function in mutant mouse strainsSherri M Jones
Department of Communication Sciences and Disorders, East Carolina University, Greenville, NC 27858, USA
J Assoc Res Otolaryngol 6:297-310. 2005..Interestingly, some heterozygote groups also showed abnormalities in one or more VsEP response parameters, suggesting that vestibular dysfunction, although less severe, may be present in some heterozygous animals... - A new spontaneous mutation in the mouse Ames waltzer gene, Pcdh15Lori L Hampton
G Protein Coupled Receptors Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
Hear Res 180:67-75. 2003..The new allele of Ames waltzer, designated Pcdh15(av-Jfb), may aid in studying the histopathology associated with Usher syndrome type 1F, which is caused by a functional null allele of PCDH15... - Inner ear proteomics of mouse models for deafness, a discovery strategyQing Yin Zheng
The Jackson Laboratory, 600 Main Street, Bar Harbor, ME 04609 1500, USA
Brain Res 1091:113-21. 2006..In this short technical report, we also discuss protein-profiling approaches suitable for SSUMM and briefly discuss other approaches used in the field of proteomics...