Combined Immunodeficiency Disease and Adenosine Deaminase Deficiency. A Molecular Defect
نویسنده
چکیده
mice are discussed by Alfred S. Steinberg who presents data in support of the idea that early loss of a thymic regulatory function in these mice may account for a breakdown in tolerance to "self" and certain other antigens, leading to excessive antibody responses with immune complex formation. The last two chapters, one by Stanley A. Plotkin, the other by Frautisek Sokol, deal, respectively, with the pros and cons of killed and live vaccines. I can recommend this book as an appetizer. It certainly whets one's appetite, but is insufficient as a meal in itself.
منابع مشابه
Adenosine deaminase deficiency and severe combined immunodeficiency disease.
The nature of the association of adenosine deaminase deficiency and severe combined immunodeficiency disease is reviewed . The basis for the molecular heterogeneity exhibited by adenosine deaminase in human tissue and the mechanisms whereby a deficiency of this activity results in the extreme perturbation of the immune system as observed in severe combined immunodeficiency are critically discus...
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Adenosine deaminase deficiency is a type of severe combined immunodeficiency that involves about 15% of all SCID cases. This form of SCID has low B cells, T cells, and NK cells. This condition is complex with many possible complications including neurological abnormalities. Guillainbarré syndrome is an acute inflammatory disease of the peripheral nerves that involves an autoimmune attack on mye...
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Adenosine deaminase deficiency accounts for approximately 15-20% of severe combined immunodeficiency in humans. The gene for adenosine deaminase is located on chromosome 20q12-q13.11 and codes for an aminohydrolase that catalyzes the deamination of adenosine and deoxyadenosine to inosine and deoxyinosine, respectively. Absence of the enzyme causes a build-up of the substrates in addition to exc...
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1. We have compared urinary purine excretion by two different methods in three separate paediatric disorders of purine metabolism: purine nucleo side phosphoryiase deficiency, adenosine deaminase deficiency and adenine phosphoribosyltransferase deficiency. 2. The abnormal purines identified in each case were specific for the defect and directly related to it: adenine in adenine phophoribosyltr...
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عنوان ژورنال:
- The Yale Journal of Biology and Medicine
دوره 49 شماره
صفحات -
تاریخ انتشار 1976