Auditory Proteomics of Mouse Model for Usher Syndrome

摘要

Approximately 30 million Americans suffer from varying degrees of hearing loss (1). In the majority of cases hearing loss is due to abnormal development or injury to sensory cells in the cochlea. Tremendous progress has been made in identifying genes linked to human hereditary impairment (HHI) in the last 10 years (2). Mouse mutants have served as an excellent model to understand the molecular pathophysiology of HHI, including those associated with Usher syndromes. Though these mouse mutants have been available for decades none of them have been utilized for proteomic studies. The aim is to identify biomarkers of sensory cells and ear pathogenesis in the Usher 1F model (3) by comparing the global protein expression changes between normal and mutant mouse cochlea.