Enzyme defects and immune dysfunction

• Record Nr.: UNISA996206967203316
• Pubbl/distr/stampa: Amsterdam ; ; New York, : Excerpta Medica, 1979
• ISBN: 1-280-78379-6; 9786613694188; 0-470-72051-4; 0-470-71819-6
• Descrizione fisica: 1 online resource (301 pages) : illustrations
• Collana: Ciba Foundation symposium. New series ; ; 68
• Disciplina: 616.3/99
• Soggetti: Immunological deficiency syndromes; Adenosine deaminase; Purines - Metabolism; Enzymes
• Lingua di pubblicazione: Inglese
• Formato: Materiale a stampa
• Livello bibliografico: Monografia
• Note generali: "Symposium on Enzyme Defects and Immune Dysfunction, held at the Ciba Foundation, London, 7th-9th November 1978"--Participants page.
• Nota di bibliografia: Includes bibliographical references and indexes.
• Nota di contenuto: Enzyme Defects and Immune Dysfunction; Contents; Chairman's introduction; Adenosine deaminase and purine nucleoside phosphorylase deficiency: how they were discovered and what they may mean; Discussion; Buridan's ass: one man's view of the immune system; Discussion; Clinical delineation of adenosine deaminase deficiency; Discussion; Immunological aberrations in purine nucleoside phosphorylase deficiencies; Discussion; Immunodeficiency due to transcobalamin II deficiency; Discussion; General Discussion I Model systems for study of enzyme deficiencies; Nucleotide metabolism in cultured T cells and in cells of patients deficient in adenosine deaminase and purine nucleoside phosphorylase Discussion; Deoxyribonucleoside toxicity in adenosine deaminase and purine nucleoside phosphorylase deficiency: implications for the development of new immunosuppressive agents; Discussion; Ecto 5'-nucleotidase deficiency in primary hypogammaglobulinaemia; Discussion; General Discussion II The methylation hypothesis of adenosine toxicity; Enzymology of immunodeficiency states; Allosteric ; regulation of calf thymus ribonucleotide reductase; Discussion; Adenosine deaminase: characteristics of the normal and mutant forms of the human enzyme Discussion; Purine nucleoside phosphorylase: the normal enzyme and structural alterations in immunodeficiency disease; Discussion; Purine nucleoside phosphorylase : immunodetection and characterization of the human enzyme; Enzyme replacement and other biochemical approaches to the therapy of adenosine deaminase deficiency; Discussion; An approach to the restoration of T cell function in a purine nucleoside phosphorylase deficient patient; Discussion; Purine metabolism in adenosine deaminase deficiency; Final General Discussion Selective treatment of lymphoid malignancy with adenosine deaminase inhibitors Defects in differentiation underlying ADA and PNP deficiency; Search for new enzyme deficiencies; Index of contributors; Subject index