The Deaf Mouse Mutant Jeff (Jf) is a Single Gene Model of Otitis Media
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چکیده
منابع مشابه
13-P138 Regulation of TGF beta signalling by Fbxo11, the gene mutated in the Jeff Otitis Media mouse mutant
lates with the maturational stage of these cells. We have studied cyclin D1 expression in the inner ear. Besides its well known function in the cell cycle control, cyclin D1 has also been implicated in other cellular processes like cell death, cellular differentiation and cancer. During early embryogenesis cyclin D1 is widely expressed in the developing sensory epithelia, but it becomes downreg...
متن کاملA mutation in the F-box gene, Fbxo11, causes otitis media in the Jeff mouse.
Otitis media (OM), inflammation of the middle ear, is the most common cause of hearing impairment and surgery in children. Recurrent and chronic forms of OM are known to have a strong genetic component, but nothing is known of the underlying genes involved in the human population. We have previously identified a novel semi-dominant mouse mutant, Jeff, in which the heterozygotes develop chronic ...
متن کاملRegulation of TGF-β signalling by Fbxo11, the gene mutated in the Jeff otitis media mouse mutant
BACKGROUND Jeff is a dominant mouse mutant displaying chronic otitis media. The gene underlying Jeff is Fbxo11, a member of the large F-box family, which are specificity factors for the SCF E3 ubiquitin ligase complex. Jeff homozygotes die shortly after birth displaying a number of developmental abnormalities including cleft palate and eyes open at birth. TGF-beta signalling is involved in a nu...
متن کاملHIF–VEGF Pathways Are Critical for Chronic Otitis Media in Junbo and Jeff Mouse Mutants
Otitis media with effusion (OME) is the commonest cause of hearing loss in children, yet the underlying genetic pathways and mechanisms involved are incompletely understood. Ventilation of the middle ear with tympanostomy tubes is the commonest surgical procedure in children and the best treatment for chronic OME, but the mechanism by which they work remains uncertain. As hypoxia is a common fe...
متن کاملPathological features in the LmnaDhe/+ mutant mouse provide a novel model of human otitis media and laminopathies.
Genetic predisposition is recognized as an important pathogenetic factor in otitis media (OM) and associated diseases. Mutant Lmna mice heterozygous for the disheveled hair and ears allele (Lmna(Dhe/+)) exhibit early-onset, profound hearing deficits and other pathological features mimicking human laminopathy associated with the LMNA mutation. We assessed the effects of the Lmna(Dhe/+) mutation ...
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ژورنال
عنوان ژورنال: Journal of the Association for Research in Otolaryngology
سال: 2002
ISSN: 1525-3961,1438-7573
DOI: 10.1007/s10162-002-3015-9