Homozygotes: A targeting vector was designed to insert a neomycin resistance (neo) cassette into exon 19 of the ATPase, Ca++ transporting, plasma membrane 2 (Atp2b2 or Pmca2) gene, abolishing gene function. Homozygous mice are viable with females being fertile. The expression of Pmca2 is tightly restricted to Purkinje neurons of the cerebellum, spiral ganglion nerves of the inner ear, and outer hair cells of the cochlea, and is required for both balance and hearing. Pmca2-/- mice have a decreased growth rate, an unsteady gait, difficulty in maintaining balance, and are deaf. They also exhibit an increased number of Purkinje neurons in the cerebellum, decreased thickness of the molecular layer, an absence of otoconia in the vestibular system, and abnormalities of the organ of Corti. These mice may be useful for studying the development of the vestibular and auditory systems, and the maintenance of otoconia.

Heterozygote: Heterozygous mice are viable, fertile, and normal in size. They exhibit normal growth, and are phenotypically similar to wildtype mice with the exception of some hearing loss.

These Atp2b2 (or Pmca2) knockout mice exhibit deafness, a decreased growth rate, an unsteady gait, an increased number of Purkinje neurons in the cerebellum, decreased thickness of the molecular layer, an absence of otoconia in the vestibular system, and abnormalities of the organ of Corti. These mice may be useful for studying the development of the vestibular and auditory systems, and the maintenance of otoconia.

This strain is hosted by NIH's MMRRC, which partners with JAX for the distribution of this and other strains. For additional information and to purchase, please visit the MMRRC site.