[Congenital hemolytic anemias].
During the last years our knowledge of the structural composition of erythrocyte membranes has markedly increased. It is the aim of this review to discuss hereditary changes of the erythrocyte membrane of pathophysiological significance on the basis of these recent biochemical findings. The report particularly deals with structural proteins, such as spectrin, and its possible significance for corpuscular deformation (e. g. spherocytosis). In addition, the importance of hemoglobinopathies and hereditary enzymatic defects for the development of hemolysis is discussed.
D000745:Anemia, Hemolytic, Congenital / Q000201:enzymology*; D004798:Enzymes / Q000172:deficiency; D004910:Erythrocyte Membrane / Q000378:metabolism; D006453:Hemoglobinopathies / Q000201:enzymology; D006455:Hemoglobins, Abnormal / Q000378:metabolism; D006801:Humans; D008560:Membrane Fluidity; D008565:Membrane Proteins / Q000378:metabolism; D013049:Spectrin / Q000378:metabolism; D013103:Spherocytosis, Hereditary / Q000201:enzymology
D004798:Enzymes; D006455:Hemoglobins, Abnormal; D008565:Membrane Proteins; D013049:Spectrin
Angeborene hämolytische Anämien.