Hemoglobin and red cell structure and function

Hemoglobin and the red cell have continued to set a dizzying pace as the objects of research in the two and one-half year interval since the First International Conference on Red Cell Metabolism and Function. Most exciting perhaps, is a beginning molecular attack on sickle cell disease. The story of the interaction of red cell metabolism and oxygen transport has continued to unfold, and we can now infer that patients with hypoxia usually utilize red cell metabolic adjustments to improve oxygenation. This puts the red cell squarely in the center of medical practice, since much of medicine-heart, pulmonary, and blood diseases - deals with inadequate oxygenation. On April 27th through the 29th, 1972, crystallographers, chemists, biochemists, physiologists, geneticists, and physicians from many medical disciplines met in the Towsley Center for Continuing Medical Education at the University of Michigan, Ann Arbor to present new data, to review recent developments, and to try to piece together additional features of the red cell puzzle. The meeting was dedicated to Dr. Francis John Worsley Roughton, Professor Emeritus of Colloid Science, University of Cambridge, England, in recognition of his numerous excellent contributions to the understanding of hemoglobin and red cell function. The program got off to a good start with a paper from M.F. Perutz, Nobel Laureate, on the structure of hemoglobin. Dr. Perutz also key-noted the Conference with a special lecture on heme-heme interaction. A number of fascinating papers were presented on various aspects of hemoglobin, its structure, its interaction with ligands such as oxygen, and its properties under varying conditions. Red cell metabolism was considered, in depth, from many viewpoints, including defects in uremia, interactions with serum phosphorous, male-female differences, the role of catalase, genetic selection for quantitative variation, and mechanisms of glycolytic response to altitude stress and to anemia. As with the first conference, a session was devoted to the continuing assessment of the importance of decline in red cell oxygen transport functional capacity during blood bank storage. A session was also devoted to consideration of carbonic anhydrase and carbon dioxide transport, and the interaction of this area with oxygen transport. A high point of the conference was the session on sickle cell structure and function. Excellent papers were presented on cyanate, including results of some early clinical trials which look promising. A trial with oral urea in sickle cell disease indicating possible usefulness of this approach was presented. The antisickling properties of carbamyl phosphate were also discussed. The present status of prenatal diagnosis of sickle cell disease, and the sickling phenomenon of deer erythrocytes, were other interesting topics. The discussions in the general area of sickle cell disease and the mechanisms by which antisickling agents act were quite interesting because of the diversity and expertise represented in the audience. This volume contains the Proceedings of this second conference. It includes the formal papers and much of the informal discussion after the papers. It represents a compilation of the present state of the art, and the status of current thinking, in the various areas discussed above.