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Faculty of Medical Sciences

Trombone : an ENU induced mouse model of late-onset deafness

Stelma, F. (Femke) (2012) Trombone : an ENU induced mouse model of late-onset deafness. thesis, Medicine.

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Abstract

Age related hearing loss, or presbycusis, is a growing problem in our ageing society and has profound effects on communication and social interaction. This multifactorial disorder is difficult to study in humans. Mouse models have given us insight in many deafness related genes. Here, a N-ethyl-N-nitrosourea (ENU) induced presbycusis mouse model, Trombone, is presented. Trombone mice show recessive hearing loss that starts in the higher frequencies and is progressive with age. The causative mutation was a T>C change in the gene Slc4a10 which causes a L617P change in the protein, a bicarbonate exchanger. Immunolocalization revealed expression in the type II and V fibrocytes of the spiral ligament, a structure that has a role in endolymph homeostasis. Trombone mice show degeneration of the neighbouring stria vascularis and profound hair cell loss in all cochlear turns. The results suggest an important role for Slc4a10 in hair cell conservation and suggest a novel role for Slc4a10 in the regulation of pH and/or endocochlear potential in the mouse inner ear.

Item Type: Thesis (Thesis)
Supervisor name: Faculty supervisor: and Dikkers, Dr. F.G. Dept of ENT
Supervisor name: External supervisor: and Bowl, Dr. Mike MRC Harwell and Second external supervisor: and Bhutta, Dr. Mahmood Nuffield Dept of Surgical Sciences
Faculty: Medical Sciences
Date Deposited: 25 Jun 2020 10:46
Last Modified: 25 Jun 2020 10:46
URI: https://umcg.studenttheses.ub.rug.nl/id/eprint/782

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