Combined immunodeficiency disease associated with adenosine deaminase deficiency

Pollara, Bernard; Pickering, R. J.; Ammann, Arthur J.; Biggar, Doug; Brunell, Philip A.; Buckley, Rebecca H.; Cohen, Flossie; Cross, V.; Dissing, J.; Giblett, E. R.; Griscelli, C; Hirschhorn, Rochelle; Hong, Richard; Huber, Jason S.; Keightley, Richard G.; Kersey, JH; Koning, J. de; Lischner, Harold W.; Los, West; Moore, Ellen; Ochs, Hans D.; Pachman, Lauren M.; Parks, Brian W.; Rosen, Fred S.; Singer, Donald H.; South, Mary Ann; Wara, Diane W.; Wolfson, Julian; Meuwissen, Hilaire J.

doi:10.1016/s0022-3476(75)80463-x

Cited by 219 publications

(48 citation statements)

References 25 publications

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“…Tissue distribution studies show that ADA is usually present in all the tissues of the body with the highest activity in lymphocytes (3) and thymocytes (4). Furthermore, congenital absence ofthis enzyme, which arises as an inborn error ofmetabolism, results in the syndrome of severe combined immunodeficiency (5)(6)(7)(8). ADA activity has been found to be subnormal in the lymphocytes of patients with chronic lymphocytic leukemia (9, 10, 11) a disease characterized by immunologic impairment and an abnormal proliferation of B cells (12).…”

Section: Introductionmentioning

confidence: 99%

Correlation of adenosine deaminase activity with cell surface markers in acute lymphoblastic leukemia.

Smyth¹,

Poplack²,

Holiman³

et al. 1978

J. Clin. Invest.

126

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Section: Introductionmentioning

confidence: 99%

Correlation of adenosine deaminase activity with cell surface markers in acute lymphoblastic leukemia.

Smyth¹,

Poplack²,

Holiman³

et al. 1978

J. Clin. Invest.

126

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“…Erythrocyte ADA deficiency has been demonstrated in about half of the cases of SLID (Meuwissen et al 1975). SLID with ADA deficiency has a great importance, because it suggests a possibility that some immunodeficiency diseases may be reflection of inborn error of metabolism.…”

Section: Discussionmentioning

confidence: 99%

“…SLID with ADA deficiency has a great importance, because it suggests a possibility that some immunodeficiency diseases may be reflection of inborn error of metabolism. Although a close relationship between ADA deficiency and SCID has been established (Hirschhorn 1975;Meuwissen et al . 1975), the role of ADA in the function of the lymphoid system cannot be defined at present.…”

Section: Discussionmentioning

confidence: 99%

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Severe Combined Immunodeficiency Disease with Adenosine Deaminase Deficiency

Yokoyama

Hayashi

Yoshimura

et al. 1979

Tohoku J. Exp. Med.

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“…The enzyme plays a vital role in the maturation of the immunological system because congenital deficiency of this enzyme is associated with Severe Combined Immunodeficiency Disease (SCID) in humans, in which both T and B cell functions are impaired [14,22]. In animals, metabolic and immunological abnormalities have been described in genetically modified mice [6].…”

mentioning

confidence: 99%

Adenosine Deaminase Activity in Cats Infected with Feline Immunodeficiency Virus

Hankanga

Kobayashi

Yamada

et al. 2007

The Journal of Veterinary Medical Science

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ABSTRACT. Adenosine deaminase (ADA), an enzyme involved in purine metabolism, has been shown to be of clinical importance in several diseases in humans. To investigate whether ADA is of any clinical significance in cats, plasma adenosine deaminase (P-ADA) and T cell adenosine deaminase (T-ADA) activities were measured in feline immunodeficiency virus (FIV) negative and positive cats. The AIDS-related complex (ARC) group showed a significant elevation in P-ADA activity compared to the asymptomatic carrier (AC), and FIV-negative groups (P<0.005). T-ADA activity was significantly elevated in FIV-positive cats compared to the FIV-negative group (P<0.05) and this elevation was attributed to the increase in the ARC group (P<0.01). A correlation was found between P-ADA and T-ADA activities in the FIV-negative group. T-ADA activity and CD4 + cell number showed a strong negative correlation in FIV-positive cats (P<0.0005). CD4 + cell numbers were significantly reduced in the ARC group compared to the healthy controls (P<0.005). Our results showed that T-ADA is increased in FIV-positive cats during the ARC stage. These results also suggest that ADA may be an indicator of T cell activation in the ARC stage of FIV infection.

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Combined immunodeficiency disease associated with adenosine deaminase deficiency

Cited by 219 publications

References 25 publications

Correlation of adenosine deaminase activity with cell surface markers in acute lymphoblastic leukemia.

Correlation of adenosine deaminase activity with cell surface markers in acute lymphoblastic leukemia.

Severe Combined Immunodeficiency Disease with Adenosine Deaminase Deficiency

Adenosine Deaminase Activity in Cats Infected with Feline Immunodeficiency Virus

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