Tabla de Contenidos:
  • 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 phosphorylaseDiscussion; 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 enzymeDiscussion; 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 inhibitorsDefects in differentiation underlying ADA and PNP deficiency; Search for new enzyme deficiencies; Index of contributors; Subject index.