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More about Dr. Thompson 101 Immunobiology and Cancer Research Program Dr. Thompson In The News
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Dr. Linda Thompson is interested in human diseases caused by mutations in DNA. She is intrigued by the idea that some diseases, such as cystic fibrosis and hemophilia, can be completely explained by a single change (i.e., mutation) in one of DNA's thousands of bases. This single base change can lead to a change in a single amino acid in a protein such as an enzyme, and this amino acid change can make the protein non-functional and cause disease. Dr. Thompson's lab studies a form of severe combined immunodeficiency caused by a mutation in the gene adenosine deaminase (ADA). Children who inherit mutations in the ADA gene lack both T and B lymphocytes, two of the most important cell types in the immune system, and will die of an infection if they do not receive a bone marrow transplant. This disease is difficult to study in the laboratory because the patients are very rare and they lack the very cells (lymphocytes) whose properties need to be investigated. Therefore, Dr. Thompson developed a model system using fetal thymic tissue from mice and a drug that can inhibit the enzyme activity of ADA and mimic the disease. She is now extending her studies using human thymic tissue that must be removed in certain cardiac surgeries so that the surgeon can have access to the heart. Another area of interest for Dr. Thompson is a second enzyme called ecto-5'-nucleotidase or 5'-NT. This enzyme produces adenosine, a substance that can bind to cell surface adenosine receptors found on almost all cells. Adenosine receptors regulate a variety of important physiological processes, such as nerve transmission, heart rate, kidney function, smooth muscle contraction, and inflammation. She and her colleagues have engineered a strain of mice that lack 5'-NT and are using these mice to understand the function of 5'-NT. Work in Dr. Thompson's lab centers on the ability of adenosine produced by 5'-NT to modulate various types of inflammatory responses. However, she has shared her mice with many other groups of scientists all over the United States and even in Germany and China so that investigators with expertise in a wide variety of fields can have access to this unique tool.
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