Literature DB >> 12943368

The deaf mouse mutant Jeff (Jf) is a single gene model of otitis media.

Rachel E Hardisty1, Alexandra Erven, Karen Logan, Susan Morse, Sylvia Guionaud, Sara Sancho-Oliver, A Jackie Hunter, Steve D M Brown, Karen P Steel.   

Abstract

Otitis media is the most common cause of hearing impairment in children and is primarily characterized by inflammation of the middle ear mucosa. Yet nothing is known of the underlying genetic pathways predisposing to otitis media in the human population. Increasingly, large-scale mouse mutagenesis programs have undertaken systematic and genome-wide efforts to recover large numbers of novel mutations affecting a diverse array of phenotypic areas involved with genetic disease including deafness. As part of the UK mutagenesis program, we have identified a novel deaf mouse mutant, Jeff (Jf). Jeff maps to the distal region of mouse chromosome 17 and presents with fluid and pus in the middle ear cavity. Jeff mutants are 21% smaller than wild-type littermates, have a mild craniofacial abnormality, and have elevated hearing thresholds. Middle ear epithelia of Jeff mice show evidence of a chronic proliferative otitis media. The Jeff mutant should prove valuable in elucidating the underlying genetic pathways predisposing to otitis media.

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Year:  2003        PMID: 12943368      PMCID: PMC3202714          DOI: 10.1007/s10162-002-3015-9

Source DB:  PubMed          Journal:  J Assoc Res Otolaryngol        ISSN: 1438-7573


  28 in total

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Journal:  Ann Otol Rhinol Laryngol       Date:  1997-08       Impact factor: 1.547

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Journal:  Laryngoscope       Date:  1997-05       Impact factor: 3.325

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Authors:  M D McGinn; D Bean-Knudsen; R W Ermel
Journal:  Hear Res       Date:  1992-04       Impact factor: 3.208

5.  Tumor necrosis factor during experimental lipopolysaccharide-induced otitis media.

Authors:  T F DeMaria; D M Murwin
Journal:  Laryngoscope       Date:  1997-03       Impact factor: 3.325

6.  Nitric oxide mediates mucin secretion in endotoxin-induced otitis media with effusion.

Authors:  A S Rose; J Prazma; S H Randell; H C Baggett; A P Lane; H C Pillsbury
Journal:  Otolaryngol Head Neck Surg       Date:  1997-03       Impact factor: 3.497

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Journal:  Laryngoscope       Date:  1994-08       Impact factor: 3.325

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Journal:  Lab Invest       Date:  1994-09       Impact factor: 5.662

9.  Shaker-1 mutations reveal roles for myosin VIIA in both development and function of cochlear hair cells.

Authors:  T Self; M Mahony; J Fleming; J Walsh; S D Brown; K P Steel
Journal:  Development       Date:  1998-02       Impact factor: 6.868

10.  Otitis media incidence and impact on the auditory brain stem response in lipopolysaccharide-nonresponsive C3H/HeJ mice.

Authors:  C R Mitchell; J B Kempton; B Scott-Tyler; D R Trune
Journal:  Otolaryngol Head Neck Surg       Date:  1997-11       Impact factor: 5.591

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  33 in total

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Authors:  Amanda H Mortensen; Qing Fang; Michelle T Fleming; Thomas J Jones; Alexandre Z Daly; Kenneth R Johnson; Sally A Camper
Journal:  Mamm Genome       Date:  2019-02-18       Impact factor: 2.957

2.  DRE-1/FBXO11-dependent degradation of BLMP-1/BLIMP-1 governs C. elegans developmental timing and maturation.

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Journal:  Dev Cell       Date:  2014-03-06       Impact factor: 12.270

3.  De Novo Variants in the F-Box Protein FBXO11 in 20 Individuals with a Variable Neurodevelopmental Disorder.

Authors:  Anne Gregor; Lynette G Sadleir; Reza Asadollahi; Silvia Azzarello-Burri; Agatino Battaglia; Lilian Bomme Ousager; Paranchai Boonsawat; Ange-Line Bruel; Rebecca Buchert; Eduardo Calpena; Benjamin Cogné; Bruno Dallapiccola; Felix Distelmaier; Frances Elmslie; Laurence Faivre; Tobias B Haack; Victoria Harrison; Alex Henderson; David Hunt; Bertrand Isidor; Pascal Joset; Satoko Kumada; Augusta M A Lachmeijer; Melissa Lees; Sally Ann Lynch; Francisco Martinez; Naomichi Matsumoto; Carey McDougall; Heather C Mefford; Noriko Miyake; Candace T Myers; Sébastien Moutton; Addie Nesbitt; Antonio Novelli; Carmen Orellana; Anita Rauch; Monica Rosello; Ken Saida; Avni B Santani; Ajoy Sarkar; Ingrid E Scheffer; Marwan Shinawi; Katharina Steindl; Joseph D Symonds; Elaine H Zackai; André Reis; Heinrich Sticht; Christiane Zweier
Journal:  Am J Hum Genet       Date:  2018-07-26       Impact factor: 11.025

Review 4.  p600/UBR4 in the central nervous system.

Authors:  Kari Parsons; Yoshihiro Nakatani; Minh Dang Nguyen
Journal:  Cell Mol Life Sci       Date:  2014-11-26       Impact factor: 9.261

5.  A hearing and vestibular phenotyping pipeline to identify mouse mutants with hearing impairment.

Authors:  Rachel E Hardisty-Hughes; Andrew Parker; Steve D M Brown
Journal:  Nat Protoc       Date:  2010-01-07       Impact factor: 13.491

Review 6.  Mouse models for the study of mucosal vaccination against otitis media.

Authors:  Albert Sabirov; Dennis W Metzger
Journal:  Vaccine       Date:  2008-02-04       Impact factor: 3.641

Review 7.  Mouse models for human otitis media.

Authors:  Dennis R Trune; Qing Yin Zheng
Journal:  Brain Res       Date:  2009-03-06       Impact factor: 3.252

Review 8.  What have we learned from murine models of otitis media?

Authors:  Hayley E Tyrer; Michael Crompton; Mahmood F Bhutta
Journal:  Curr Allergy Asthma Rep       Date:  2013-10       Impact factor: 4.806

9.  Eya4-deficient mice are a model for heritable otitis media.

Authors:  Frederic F S Depreux; Keith Darrow; David A Conner; Roland D Eavey; M Charles Liberman; Christine E Seidman; J G Seidman
Journal:  J Clin Invest       Date:  2008-02       Impact factor: 14.808

10.  Regulation of TGF-beta signalling by Fbxo11, the gene mutated in the Jeff otitis media mouse mutant.

Authors:  Hilda Tateossian; Rachel E Hardisty-Hughes; Susan Morse; Maria R Romero; Helen Hilton; Charlotte Dean; Steve Dm Brown
Journal:  Pathogenetics       Date:  2009-07-06
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