Article
Life and death of sensory hair cells expressing constitutively active TRPML3.
The Journal of biological chemistry - 15 May 2009
Grimm Christian, Jörs Simone, Heller Stefan
Abstract excerpt
The varitint-waddler mutation A419P renders TRPML3 constitutively active, resulting in cationic overload, particularly in sustained influx of Ca(2+). TRPML3 is expressed by inner ear sensory hair cells, and we were intrigued by the fact that hair cells are able to cope with expressing the TRPML3(A419P) isoform for weeks before they ultimately die. We hypothesized that the survival of varitint-waddler hair cells...
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