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

The Journal of Clinical Investigation
R HirschhornP 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.

References

Jan 1, 1978·Proceedings of the National Academy of Sciences of the United States of America·A CohenD W Martin
Oct 1, 1978·The Journal of Pediatrics·F C SchmalstiegR M Goldblum
Feb 22, 1979·The New England Journal of Medicine·A RubinsteinR A Murphy
Oct 1, 1979·The Journal of Clinical Investigation·R HirschhornW Borkowsky
Dec 9, 1976·The New England Journal of Medicine·S H PolmarR Hirschhorn
Aug 1, 1976·Proceedings of the National Academy of Sciences of the United States of America·G C MillsR M Goldblum
Apr 3, 1975·The New England Journal of Medicine·R ParkmanR Hirschhorn
Feb 18, 1969·Biochimica Et Biophysica Acta·A W Solter, R E Handschumacher

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Citations

Jan 1, 1991·Journal of Inherited Metabolic Disease·E TakedaM Miyao
Jun 1, 1994·Journal of Biomedical Science·I.K. KimP.K. Chiang
Sep 1, 1985·Biochemical Pharmacology·F W BurgessR E Parks
Apr 15, 1986·Clinica Chimica Acta; International Journal of Clinical Chemistry·H A BucJ L Pérignon
Dec 30, 1988·Clinica Chimica Acta; International Journal of Clinical Chemistry·R BoulieuG Souillet
Jan 1, 1982·The International Journal of Biochemistry·G J PetersJ H Veerkamp
Jan 10, 1991·Biochimica Et Biophysica Acta·D A CarsonT Iizasa
Nov 9, 1990·Biochimica Et Biophysica Acta·H D Kim
Nov 1, 2001·Biochimica Et Biophysica Acta·M C CartaF Sgarrella
Jan 5, 1999·Bioorganic & Medicinal Chemistry Letters·M A CurtisE Abushanab
Aug 14, 1980·The New England Journal of Medicine·R HirschhornL T Taft
Oct 1, 1980·Proceedings of the National Academy of Sciences of the United States of America·M F SiawJ J Hutton
Jun 1, 1982·Proceedings of the National Academy of Sciences of the United States of America·D A CarsonN Kamatani
Oct 1, 1983·The Journal of Experimental Medicine·B N CronsteinR Hirschhorn
Jan 19, 2008·Journal of Nutritional Science and Vitaminology·Shigeko Fujimoto SakataNanaya Tamaki
Feb 8, 2005·The International Journal of Biochemistry & Cell Biology·Michela GiannecchiniMarcella Camici
Apr 2, 2016·Proceedings of the National Academy of Sciences of the United States of America·Woosuk KimCaius G Radu
Oct 19, 2013·Current Opinion in Allergy and Clinical Immunology·Emmanuelle JouanguyJean-Laurent Casanova
Jan 1, 1982·Cancer Chemotherapy and Pharmacology·J F Kuttesch, J A Nelson
Jul 28, 1995·The Journal of Biological Chemistry·A R WakadeT D Wakade
Mar 5, 1987·The New England Journal of Medicine·M S HershfieldA L Kobayashi
Feb 24, 2011·Expert Review of Anti-infective Therapy·Melanie R Power CoombsOfer Levy
Dec 1, 1981·The Journal of Dermatology·Y P KimC W Ihm
Dec 29, 2020·Annals of Allergy, Asthma & Immunology : Official Publication of the American College of Allergy, Asthma, & Immunology·Melissa D GansArye Rubinstein
Feb 11, 2018·Biochemical Pharmacology·György HaskóBruce N Cronstein
May 11, 1984·Journal of Chromatography·J Ontyd, J Schrader
Apr 15, 1982·Biochemical Pharmacology·C M Smith, J F Henderson
Jun 1, 1981·Metabolism: Clinical and Experimental·I H Fox

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