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Adenosine deaminase deficiency

Adenosine deaminase deficiency, or ADA deficiency, is an inherited immunodeficiency syndrome accounting for about 25% of all cases of severe combined immunodeficiency (SCID). more...

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This disease is due to a lack of the enzyme adenosine deaminase coded for by a gene on chromosome 20. There is an accumulation of dATP, which causes an increase in S-adenosylhomocysteine; both substances are toxic to immature lymphoid cells, so fail to reach maturity. As a result, the immune system of the afflicted person is severely compromised or completely lacking.

The enzyme adenosine deaminase is important for purine metabolism.

Treatment

  • bone marrow transplant
  • gene therapy (efforts halted due to increased incidence of leukemia)
  • ADA enzyme in PEG vehicle

The first gene therapy to combat this disease was performed by Dr. W. French Anderson on a 4yr old girl, Ashanti DeSilva, in 14 September 1990 at the National Institute of Health, Bethesda, Maryland, U.S.A.

The therapy performed was the first successful case of gene therapy.

Read more at Wikipedia.org


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Enzyme ruse successful - method to protect adenosine deaminase from destruction by immune system
From Science News, 3/7/87

Enzyme ruse successful

A method of protecting a therapeutic enzyme from destructionby the immune system has proven "remarkably successful,' says Rebecca H. Buckley of Duke University in Durham, N.C. She and her colleagues used the treatment earlier this year (SN: 5/3/86, p.277) to supply the enzyme, adenosine deaminase (ADA), to two children with severe combined immunodeficiency.

The children had been born without the ability to producetheir own ADA. Without ADA, the immune system can't function normally, but it can still destroy foreign ADA.

Buckley and her colleagues used ADA molecules studdedwith polyethylene glycol (PEG), the main ingredient in antifreeze. The PEG shielded the enzyme from immune system cells, but the smaller molecules on which the enzyme normally acts were able to diffuse through the PEG barrier. The ADA brought back immune function where there had been none, Buckley reported at the meeting and in the March 5 NEW ENGLAND JOURNAL OF MEDICINE.

The only other treatment for ADA deficiency is bone marrowtransplantation, for which a matched donor is needed and which is effective in only half the cases treated. The antifreeze protection may work in other enzyme deficiencies as well, Buckley says.

COPYRIGHT 1987 Science Service, Inc.
COPYRIGHT 2004 Gale Group

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