
pmid: 29910743
pmc: PMC5992676
Deformability is an essential feature of blood cells (RBCs) that enables them to travel through even the smallest capillaries of the human body. Deformability is a function of (i) structural elements of cytoskeletal proteins, (ii) processes controlling intracellular ion and water handling and (iii) membrane surface-to-volume ratio. All these factors may be altered in various forms of hereditary hemolytic anemia, such as sickle cell disease, thalassemia, hereditary spherocytosis and hereditary xerocytosis. Although mutations are known as the primary causes of these congenital anemias, little is known about the resulting secondary processes that affect RBC deformability (such as secondary changes in RBC hydration, membrane protein phosphorylation, and RBC vesiculation). These secondary processes could, however, play an important role in the premature removal of the aberrant RBCs by the spleen. Altered RBC deformability could contribute to disease pathophysiology in various disorders of the RBC. Here we review the current knowledge on RBC deformability in different forms of hereditary hemolytic anemia and describe secondary mechanisms involved in RBC deformability.
thalassemia, Physiology, Hydration, 610, Hereditary spherocytosis, Hemolysis, enzymopathies, Enzymopathies, 2737 Physiology (medical), sickle cell anemia, 11554 Zurich Center for Integrative Human Physiology (ZIHP), Physiology (medical), QP1-981, Deformability, Vesiculation, deformability, hereditary spherocytosis, ddc:610, 1314 Physiology, 10081 Institute of Veterinary Physiology, vesiculation, Sickle cell anemia, 570 Life sciences; biology, Thalassemia, hemolysis, hydration
thalassemia, Physiology, Hydration, 610, Hereditary spherocytosis, Hemolysis, enzymopathies, Enzymopathies, 2737 Physiology (medical), sickle cell anemia, 11554 Zurich Center for Integrative Human Physiology (ZIHP), Physiology (medical), QP1-981, Deformability, Vesiculation, deformability, hereditary spherocytosis, ddc:610, 1314 Physiology, 10081 Institute of Veterinary Physiology, vesiculation, Sickle cell anemia, 570 Life sciences; biology, Thalassemia, hemolysis, hydration
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