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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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Gene therapy proposal gains acceptance - to treat children with adenosine deaminase deficiency
From Science News, 6/16/90

Gene therapy proposal gains acceptance

On June 1, a National Institutes of Health (NIH) subcommittee approved use of an experimental gene therapy to treat children with a severe, inherited immune disorder. The project must clear other regulatory hurdles, but federal scientists say they may begin treating three or four children with adenosine deaminase deficiency as early as this fall.

Children afflicted with this disorder lack the enzyme adenosine deaminase, a problem that results in destruction of white cells that help the body fight infection. Under the plan, scientists would engineer white cells to carry healthy genes that code for the missing enzyme (SN: 4/7/90, p.213).

NIH researchers R. Michael Blaese and W. French Anderson note that their proposal must first win the approval of another NIH review panel and the Food and Drug Administration.

COPYRIGHT 1990 Science Service, Inc.
COPYRIGHT 2004 Gale Group

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