Disruption of the adenosine deaminase gene causes hepatocellular impairment and perinatal lethality in mice

Maki Wakamiya; Michael R. Blackburn; Roland Jurecic; Mark J. McArthur; Robert S. Geske; Joiner Cartwright; Kohnosuke Mitani; Sukeshi Vaishnav; John W. Belmont; Rodney E. Kellems; Milton J. Finegold; Charles A. Montgomery; Allan Bradley; C. Thomas Caskey

doi:10.1073/pnas.92.9.3673

Disruption of the adenosine deaminase gene causes hepatocellular impairment and perinatal lethality in mice

Maki Wakamiya, Michael R. Blackburn, Roland Jurecic, Mark J. McArthur, Robert S. Geske, Joiner Cartwright, Kohnosuke Mitani, Sukeshi Vaishnav, John W. Belmont, Rodney E. Kellems, Milton J. Finegold, Charles A. Montgomery, Allan Bradley, C. Thomas Caskey

Veterinary Medicine & Surgery

Research output: Contribution to journal › Article › peer-review

127 Scopus citations

Abstract

We have generated mice with a null mutation at the Ada locus, which encodes the purine catabolic enzyme adenosine deaminase (ADA, EC 3.5.4.4). ADA-deficient fetuses exhibited hepatocellular impairment and died perinatally. Their lymphoid tissues were not largely affected. Accumulation of ADA substrates was detectable in ADA-deficient conceptuses as early as 12.5 days postcoitum, dramatically increasing during late in utero development, and is the likely cause of liver damage and fetal death. The results presented here demonstrate that ADA is important for the homeostatic maintenance of purines in mice.

Original language	English (US)
Pages (from-to)	3673-3677
Number of pages	5
Journal	Proceedings of the National Academy of Sciences of the United States of America
Volume	92
Issue number	9
DOIs	https://doi.org/10.1073/pnas.92.9.3673
State	Published - Apr 25 1995

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Wakamiya, M., Blackburn, M. R., Jurecic, R., McArthur, M. J., Geske, R. S., Cartwright, J., Mitani, K., Vaishnav, S., Belmont, J. W., Kellems, R. E., Finegold, M. J., Montgomery, C. A., Bradley, A., & Caskey, C. T. (1995). Disruption of the adenosine deaminase gene causes hepatocellular impairment and perinatal lethality in mice. Proceedings of the National Academy of Sciences of the United States of America, 92(9), 3673-3677. https://doi.org/10.1073/pnas.92.9.3673

Wakamiya, M, Blackburn, MR, Jurecic, R, McArthur, MJ, Geske, RS, Cartwright, J, Mitani, K, Vaishnav, S, Belmont, JW, Kellems, RE, Finegold, MJ, Montgomery, CA, Bradley, A & Caskey, CT 1995, 'Disruption of the adenosine deaminase gene causes hepatocellular impairment and perinatal lethality in mice', Proceedings of the National Academy of Sciences of the United States of America, vol. 92, no. 9, pp. 3673-3677. https://doi.org/10.1073/pnas.92.9.3673

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abstract = "We have generated mice with a null mutation at the Ada locus, which encodes the purine catabolic enzyme adenosine deaminase (ADA, EC 3.5.4.4). ADA-deficient fetuses exhibited hepatocellular impairment and died perinatally. Their lymphoid tissues were not largely affected. Accumulation of ADA substrates was detectable in ADA-deficient conceptuses as early as 12.5 days postcoitum, dramatically increasing during late in utero development, and is the likely cause of liver damage and fetal death. The results presented here demonstrate that ADA is important for the homeostatic maintenance of purines in mice.",

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AU - Geske, Robert S.

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AU - Mitani, Kohnosuke

AU - Vaishnav, Sukeshi

AU - Belmont, John W.

AU - Kellems, Rodney E.

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AU - Caskey, C. Thomas

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AB - We have generated mice with a null mutation at the Ada locus, which encodes the purine catabolic enzyme adenosine deaminase (ADA, EC 3.5.4.4). ADA-deficient fetuses exhibited hepatocellular impairment and died perinatally. Their lymphoid tissues were not largely affected. Accumulation of ADA substrates was detectable in ADA-deficient conceptuses as early as 12.5 days postcoitum, dramatically increasing during late in utero development, and is the likely cause of liver damage and fetal death. The results presented here demonstrate that ADA is important for the homeostatic maintenance of purines in mice.

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Disruption of the adenosine deaminase gene causes hepatocellular impairment and perinatal lethality in mice

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