20. Adenosine deaminase deficiency results in a 100-fold increase in the cellula
ID: 696547 • Letter: 2
Question
20. Adenosine deaminase deficiency results in a 100-fold increase in the cellular concentration of dATP. Using knowledge gained in chapter 18 and thinking carefully, predict the physiological effect of the elevated concentration of dATP 21. Glutamine synthetase is the key regulatory enzyme in the assimilation of ammonia and is thus very highly regulated. The enzyme from Escherichia coli is regulated by allosteric and covalent means. Explain the regulation of glutamine synthetase in E. coli (you may wish to use a diagram)Explanation / Answer
ADA or adenosine deaminase deficiency could lead to an elevated concentration of ribonucleotide reductase inhibitor-dATP.,which further inhibits synthesis of DNA.,thereby,stopping cell division.The actively dividing T-cells and B-cells are therefore prevented from cell division,
Thus,an elevated dATP causes apoptosis or destruction of thymocytes(lymphocyte in thymus) or the T-cells.These thymocytes are responsible for immune system development and functioning which stands impaired on thymocytes apoptosis.
Also the B-cell(or bone-marrow cell)deficiency causes severe immune deficiency as antibodies production is hampered.
Other effects of dATP include neurological issues like mental retardation.Also,an increased adenosine signaling causing surfactant accumulation on lungs,or,PAP(pulmonary alveolar proteinosis) causing lung dysfunction and breathing problems.
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