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TRPML3, a member of the transient receptor potential (TRP) family, is an inwardly rectifying, non-selective Ca 2+ -permeable cation channel that is regulated by extracytosolic Na +  and H +  and can be activated by a variety of small molecules. The severe auditory and vestibular phenotype of the TRPML3(A419P) varitint-waddler mutation made this protein particularly interesting for inner ear biology. To elucidate the physiological role of murine TRPML3, we conditionally inactivated  Trpml3  in mice. Surprisingly, lack of functional TRPML3 did not lead to circling behavior, balance impairment or hearing loss.

Genetic Inactivation of Trpml3 Does Not Lead to Hearing and Vestibular Impairment in Mice