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DFNA5: hearing impairment exon instead of hearing impairment gene?
Journal article   Open access   Peer reviewed

DFNA5: hearing impairment exon instead of hearing impairment gene?

L Van Laer, K Vrijens, S Thys, V F I Van Tendeloo, R Smith, D R Van Bockstaele, J Timmermans and G Van Camp
Journal of medical genetics, Vol.41(6), pp.401-406
06/2004
DOI: 10.1136/jmg.2003.015073
PMCID: PMC1735793
PMID: 15173223
url
https://doi.org/10.1136/jmg.2003.015073View
Published (Version of record) Open Access

Abstract

Methods: We performed transfection experiments in mammalian cell lines (HEK293T and COS-1) with green fluorescent protein (GFP) tagged wildtype and mutant DFNA5 and analysed cell death with flow cytometry and fluorescence microscopy. Results: Post-transfection death of HEK293T cells approximately doubled when cells were transfected with mutant DFNA5 –GFP compared with wildtype DFNA5–GFP. Cell death was attributed to necrotic events and not to apoptotic events. Conclusion: The transfection experiments in mammalian cell lines support our hypothesis that the hearing impairment associated with DFNA5 is caused by a "gain of function" mutation and that mutant DFNA5 has a deleterious new function.
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