Diaphanous homolog 3 is a member of the diaphanous-related formin protein family which are involved in actin regulation and microtubule stabilization. A mutation in human DIAPH3 is a cause of autosomal dominant auditory neuropathy. These transgenic mice overexpress the mouse Diap3, diaphanous homolog 3 (Drosophila), gene under the control of the CAG, CMV enhancer/chicken beta-actin promoter, with approximately 1000-fold overexpression of Diap3 in the cochlea, as determined by qRT-PCR. Hemizygotes develop hearing loss between 8 and 16 weeks of age, which progresses to severe hearing loss by 24 weeks of age.
Hemizygotes have fewer inner hair cell ribbon synapses and disorganized inner hair cell stereocilia. Some transgenic mice die prematurely due to cardiac abnormalities (enlarged hearts, cardiomyopathy). Mice hemizygous for the transgenic insert are viable and fertile. The Donating Investigator has not attempted to make the strain homozygous.

These transgenic mice overexpress mouse Diap3, diaphanous homolog 3 (Drosophila), in the cochlea, exhibit progressive hearing loss and may be useful in studies of progressive hearing loss and auditory neuropathy.

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FVB/NJ-Tg(CAG-Diap3)924Lesp/J Frozen Embryo