Skip to main content
ARS Home » Midwest Area » Ames, Iowa » National Animal Disease Center » Ruminant Diseases and Immunology Research » Research » Publications at this Location » Publication #166160
Title: THE GENETIC IMMUNODEFICIENCY DISEASE LEUKOCYTE ADHESION DEFICIENCY IN HUMANS, DOGS, CATTLE, AND MICE

Author
item GU, YU-CHEN - NATIONAL INST OF HEALTH
item BAUER JR, THOMAS - NATIONAL INST OF HEALTH
item ACKERMANN, M - IOWA STATE UNIVERSITY
item SMITH, C - BAYLOR COLLEGE MEDICINE
item Kehrli Jr, Marcus
item STAROST, M - NATIONAL INST OF HEALTH
item HICKSTEIN, D - NATIONAL INST OF HEALTH

Submitted to: Comparative Medicine
Publication Type: Peer Reviewed Journal
Publication Acceptance Date: 5/19/2004
Publication Date: 8/1/2004
Citation: Gu, Y., Bauer Jr, T.R., Ackermann, M.R., Smith, C.W., Kehrli Jr, M.E., Starost, M.F., Hickstein, D.D. 2004. The genetic immunodeficiency disease leukocyte adhesion deficiency in humans, dogs, cattle, and mice. Comparative Medicine. 54(4):363-72.

Interpretive Summary: Dairy cattle die as a result of many diseases. This was estimated to cost $75.5 million annually in the United States. The majority of this loss can be categorized into deaths due to pneumonia and diarrhea. We have previously identified a genetic disorder in Holstein dairy cows that makes calves susceptible to fatal cases of pneumonia and diarrhea. The research reported here provides evidence that bone marrow transplants resulting in only a few fully functional, circulating leukocytes can provide sufficient defense against disease and maintain quite healthy animals well into adult life. To reach this conclusion, we studied for over 5 years a set of non-identical twin calves with a naturally-occurring bone marrow transplant that takes place during pregnancy as a result of a shared blood supply between twins. The information reported here provides a basis for clinicians and researchers to seek out less aggressive approaches to "wipe out" the defective immune system of children with "leukocyte adhesion deficiency" prior to bone marrow transplantation. An additional benefit of this of report is that we have demonstrated this as a model for studying methods of immune function restoration in humans with leukocyte adhesion deficiency.

Technical Abstract: This review highlights the genotype-phenotype relationship of the genetic immunodeficiency disease leukocyte adhesion deficiency (LAD) in humans, dogs, cattle, and mice, and provides assessment of the opportunities that each animal species provides in the understanding of leukocyte biology and in developing new therapeutic approaches to LAD in humans. This comparison is important since animal models of genetic diseases in humans provide the opportunity to test new therapeutic approaches in an appropriate, disease-specific model. The success of this approach is dependent on the relationship of the phenotype in the animal to the phenotype of the disease in humans.