Mice homozygous for the Adatm1Mw targeted mutation die perinatally. They show defects in purine metabolism and have liver cell degeneration. Death is most likely the result of accumulation of ADA precursors. Mice from the double mutant strain FVB;129- Adatm1Mw-TgN(PLADA)4118Rkmb/J (Stock No. <a href="https://www.jax.org/strain/003265">003265</a>) are rescued from embryonic lethality by transgenic ADA expression in the placenta. Rescued mice that are homozygous for the null Ada allele exhibit a severe combined immunodeficiency. In addition, mice develop a severe lung eosinopilia reminescent of that seen in humans with asthma. Abnormalities were also found in the bone and kidney. ADA deficient mice die from severe respiratory distress by three weeks of age. Mice carrying a transgene overexpressiong ADA in both the placenta and forestomach, FVB;129- Adatm1Mw-TgN(PLFSADA)2465Rkmb/J (Stock No. <a href="https://www.jax.org/strain/003297">003297</a>), are rescued from postnatal lethality at three weeks of age. Rescued mice that are homozygous for the null Ada allele live a normal lifespan displaying only a partial immune deficiency and developing less severe pulmonary inflammation.

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These Ada knock-out mice exhibit perinatal lethality with and liver cell degeneration and defects in purine metabolism. They may be suitable for use in applications related to the study of severe combined immune deficiency in humans.

Typically mice are recovered in 12-16 weeks. Contact Customer Service to place an order or for more information.

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