Literature DB >> 6965496

Plasma deoxyadenosine, adenosine, and erythrocyte deoxyATP are elevated at birth in an adenosine deaminase-deficient child.

R Hirschhorn, V Roegner, A Rubinstein, P Papageorgiou.   

Abstract

We have determined concentrations of adenosine, deoxyadenosine, and deoxyATP (dATP) in cord blood from an infant prenatally diagnosed as ADA deficient. Plasma deoxyadenosine and adenosine were already elevated in cord blood (0.7 and 0.5 microM vs. normal of less than 0.07 microM). Elevation of plasma deoxyadenosine has not previously been documented in these children. Erythrocyte dATP content was also elevated at birth (215 nmol/ml packed erythrocytes vs. normal of 2.9). These elevated concentrations of adenosine, deoxyadenosine, and dATP are similar to those we observed in another older adenosine deaminase-deficient patient and may explain the impaired immune function and lymphopenia seen at birth.

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Year:  1980        PMID: 6965496      PMCID: PMC371421          DOI: 10.1172/JCI109725

Source DB:  PubMed          Journal:  J Clin Invest        ISSN: 0021-9738            Impact factor:   14.808


  15 in total

1.  Adenosine-deaminase deficiency in two patients with severely impaired cellular immunity.

Authors:  E R Giblett; J E Anderson; F Cohen; B Pollara; H J Meuwissen
Journal:  Lancet       Date:  1972-11-18       Impact factor: 79.321

2.  A rapid quantitative determination of deoxyribonucleoside triphosphates based on the enzymatic synthesis of DNA.

Authors:  A W Solter; R E Handschumacher
Journal:  Biochim Biophys Acta       Date:  1969-02-18

3.  Combined immunodeficiency disease associated with adenosine deaminase deficiency. Report on a workshop held in Albany, New York, October 1, 1973.

Authors:  H J Meuwissen; B Pollara; R J Pickering
Journal:  J Pediatr       Date:  1975-02       Impact factor: 4.406

4.  Severe combined immunodeficiency and adenosine deaminase deficiency.

Authors:  R Parkman; E W Gelfand; F S Rosen; A Sanderson; R Hirschhorn
Journal:  N Engl J Med       Date:  1975-04-03       Impact factor: 91.245

5.  Purine metabolism and immunodeficiency: urinary purine excretion as a diagnostic screening test in adenosine deaminase and purine nucleoside phosphorylase deficiency.

Authors:  H A Simmonds; A Sahota; C F Potter; J S Cameron
Journal:  Clin Sci Mol Med       Date:  1978-05

6.  In vivo and in vitro effects of thymosin and adenosine deaminase on adenosine-deaminase-deficient lymphocytes.

Authors:  A Rubinstein; R Hirschhorn; M Sicklick; R A Murphy
Journal:  N Engl J Med       Date:  1979-02-22       Impact factor: 91.245

7.  Restoration of in-vitro lymphocyte responses with exogenous adenosine deaminase in a patient with severe combined immunodeficiency.

Authors:  S H Polmar; E M Wetzler; R C Stern; R Hirschhorn
Journal:  Lancet       Date:  1975-10-18       Impact factor: 79.321

8.  Adenosine deaminase deficiency: disappearance of adenine deoxynucleotides from a patient's erythrocytes after successful marrow transplantation.

Authors:  S H Chen; H D Ochs; C R Scott; E R Giblett; A J Tingle
Journal:  J Clin Invest       Date:  1978-12       Impact factor: 14.808

9.  Purine metabolism in adenosine deaminase deficiency.

Authors:  G C Mills; F C Schmalstieg; K B Trimmer; A S Goldman; R M Goldblum
Journal:  Proc Natl Acad Sci U S A       Date:  1976-08       Impact factor: 11.205

10.  Erythrocyte adenosine deaminase deficiency without immunodeficiency. Evidence for an unstable mutant enzyme.

Authors:  R Hirschhorn; V Roegner; T Jenkins; C Seaman; S Piomelli; W Borkowsky
Journal:  J Clin Invest       Date:  1979-10       Impact factor: 14.808

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  19 in total

Review 1.  Adenosine metabolism, immunity and joint health.

Authors:  György Haskó; Luca Antonioli; Bruce N Cronstein
Journal:  Biochem Pharmacol       Date:  2018-02-07       Impact factor: 5.858

2.  Adenosine modulates Toll-like receptor function: basic mechanisms and translational opportunities.

Authors:  Melanie R Power Coombs; Mirjam E Belderbos; Leighanne C Gallington; Louis Bont; Ofer Levy
Journal:  Expert Rev Anti Infect Ther       Date:  2011-02       Impact factor: 5.091

3.  Homozygosity for a newly identified missense mutation in a patient with very severe combined immunodeficiency due to adenosine deaminase deficiency (ADA-SCID).

Authors:  R Hirschhorn; V Chakravarti; J Puck; S D Douglas
Journal:  Am J Hum Genet       Date:  1991-10       Impact factor: 11.025

4.  Pathologic findings in adenosine deaminase-deficient severe combined immunodeficiency. I. Kidney, adrenal, and chondro-osseous tissue alterations.

Authors:  H Ratech; M A Greco; G Gallo; D L Rimoin; H Kamino; R Hirschhorn
Journal:  Am J Pathol       Date:  1985-07       Impact factor: 4.307

Review 5.  Metabolic defects in immunodeficiency diseases.

Authors:  A D Webster
Journal:  Clin Exp Immunol       Date:  1982-07       Impact factor: 4.330

6.  A new procedure for haptenizing adenosine leading to a more specific radioimmunoassay method.

Authors:  A C Newby; G B Sala
Journal:  Biochem J       Date:  1982-12-15       Impact factor: 3.857

7.  Biochemical and functional abnormalities in lymphocytes from an adenosine deaminase-deficient patient during enzyme replacement therapy.

Authors:  J J Hutton; D A Wiginton; M S Coleman; S A Fuller; S Limouze; B C Lampkin
Journal:  J Clin Invest       Date:  1981-08       Impact factor: 14.808

8.  Renal handling of 2'-deoxyadenosine and adenosine in humans and mice.

Authors:  J F Kuttesch; J A Nelson
Journal:  Cancer Chemother Pharmacol       Date:  1982       Impact factor: 3.333

9.  Altered intracellular and extracellular signaling leads to impaired T-cell functions in ADA-SCID patients.

Authors:  Barbara Cassani; Massimiliano Mirolo; Federica Cattaneo; Ulrike Benninghoff; Michael Hershfield; Filippo Carlucci; Antonella Tabucchi; Claudio Bordignon; Maria Grazia Roncarolo; Alessandro Aiuti
Journal:  Blood       Date:  2008-01-24       Impact factor: 22.113

10.  Genetic heterogeneity in adenosine deaminase (ADA) deficiency: five different mutations in five new patients with partial ADA deficiency.

Authors:  R Hirschhorn; A Ellenbogen
Journal:  Am J Hum Genet       Date:  1986-01       Impact factor: 11.025

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