ADENOSINE DEAMINASE /ADA/ AND PURINE METABOLISM IN HUMAN LYMPHOCYTES
نویسندگان
چکیده
منابع مشابه
Purine metabolism in adenosine deaminase deficiency.
Deoxyadenosine was identified in the urine of a second child with almost undetectable levels of adenosine deaminase (ADA) in erythrocyte lysates. Deoxyadenosine excretion thus appears to be characteristic of ADA deficiency: the acid lability of deoxyadenosine (responsible for the frequent confusion of this abnormal urinary metabolite with adenine) may be used in screening for this defect by iso...
متن کاملPurine Nucleoside Phosphorylase ( PNP ) and Adenosine Deaminase ( ADA ) Activities
New techniques have been devised for the cytochemical demonstration of purine nucleoside phosphorylase (PNP) and adenosine deaminase (ADA) activities in unfixed human lymphocytes. A suspension of living lymphocytes is mixed with agarose sol containing the reagents for the detection of PNP or ADA activity on a glass slide. The mixture solidifies. is incubated, and then dried for lightmicroscopic...
متن کاملAutoimmune Dysregulation and Purine Metabolism in Adenosine Deaminase Deficiency
Genetic defects in the adenosine deaminase (ADA) gene are among the most common causes for severe combined immunodeficiency (SCID). ADA-SCID patients suffer from lymphopenia, severely impaired cellular and humoral immunity, failure to thrive, and recurrent infections. Currently available therapeutic options for this otherwise fatal disorder include bone marrow transplantation (BMT), enzyme repl...
متن کاملPurine nucleoside phosphorylase (PNP) and adenosine deaminase (ADA) activities examined cytochemically in unfixed lymphocytes of patients with lymphoproliferative disorders.
New techniques have been devised for the cytochemical demonstration of purine nucleoside phosphorylase (PNP) and adenosine deaminase (ADA) activities in unfixed human lymphocytes. A suspension of living lymphocytes is mixed with agarose sol containing the reagents for the detection of PNP or ADA activity on a glass slide. The mixture solidifies, is incubated, and then dried for lightmicroscopic...
متن کاملPurine metabolism in cultured human fibroblasts derived from patients deficient in hypoxanthine phosphoribosyltransferase, purine nucleoside phosphorylase, or adenosine deaminase.
Rates of purine synthesis de novo, as measured by the incorporation of [14C]formate into newly synthesized purines, have been determined in cultured human fibroblasts derived from normal individuals and from patients deficient in adenosine deaminase, purine nucleoside phosphorylase, or hypoxanthine phosphoribosyltransferase, three consecutive enzymes of the purine salvage pathway. All four type...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1975
ISSN: 0031-3998
DOI: 10.1203/00006450-197509110-00106