adenosine deaminase deficiency

a genetic disorder affecting about one baby in 25,000 and characterized by a defect in adenosine deaminase (ADA), an enzyme that is involved in purine metabolism. Deficiency of this enzyme results in selective damage to the antibody-producing lymphocytes; this in turn leads to a condition known as severe combined immune deficiency (SCID), in which the affected baby has no resistance to infection and must be entirely isolated from birth. Such children have only about a 50% chance of surviving for six months. See also gene therapy.

Medical dictionary. 2011.

Look at other dictionaries:

  • Adenosine deaminase deficiency — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 260 ICD10 = ICD10|D|81|3|d|80 ICD9 = ICD9|279.2 ICDO = OMIM = 102700 MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = Adenosine deaminase deficiency, also called ADA deficiency or ADA… …   Wikipedia

  • adenosine deaminase deficiency — ADA deficiency a genetic disorder affecting about one baby in 25,000 and characterized by a defect in adenosine deaminase (ADA), an enzyme that is involved in purine metabolism. Deficiency of this enzyme results in selective damage to the… …   The new mediacal dictionary

  • adenosine deaminase deficiency — a severe immune system disorder caused by a genetic inability to produce adenosine deaminase. Also called ADA deficiency. * * * …   Universalium

  • adenosine deaminase deficiency — a severe immune system disorder caused by a genetic inability to produce adenosine deaminase. Also called ADA deficiency …   Useful english dictionary

  • Дефицит Аденозин Дезаминазы (Ада Дефицит) (Adenosine Deaminase Deficiency, Ada Deficiency) — наследственное заболевание, встречающееся у одного из каждых 25 000 младенцев и характеризующееся дефицитом содержания в организме аденозин дезаминазы (АДА) (adenosine deaminase (ADA)), фермента, участвующего в метаболизме пуринов. Дефицит… …   Медицинские термины

  • Adenosine Monophosphate Deaminase Deficiency type 1 — Classification and external resources Adenosine monophosphate OMIM 102770 …   Wikipedia

  • Adenosine deaminase — (also known as ADA) is an enzyme (EC number|3.5.4.4) involved in purine metabolism. It is needed for the breakdown of adenosine from food and for the turnover of nucleic acids in tissues.ReactionsADA irreversibly deaminates adenosine, converting… …   Wikipedia

  • adenosine deaminase — n an enzyme which catalyzes the conversion of adenosine to inosine and whose deficiency causes a form of severe combined immunodeficiency disease as a result of the accumulation of toxic metabolites which inhibit DNA synthesis abbr. ADA * * *… …   Medical dictionary

  • Adenosine deaminase (ADA) deficiency — A genetic (inherited) condition that results in a immune deficiency disorder called severe combined immunodeficiency disease. Adenosine deaminase is an enzyme that plays a key role in salvaging purine molecules. ADA deficiency is of special… …   Medical dictionary

  • Deficiency, adenosine deaminase (ADA) — A genetic (inherited) condition that results in a immune deficiency disorder called severe combined immunodeficiency disease. Adenosine deaminase is an enzyme that plays a key role in salvaging purine molecules. ADA deficiency is of special… …   Medical dictionary

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