Squeezing for Life - Properties of Red Blood Cell Deformability.
- Huisjes R Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.
- Bogdanova A Red Blood Cell Research Group, Institute of Veterinary Physiology, Vetsuisse Faculty and the Zurich Center for Integrative Human Physiology (ZIHP), University of Zurich, Zürich, Switzerland.
- van Solinge WW Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.
- Schiffelers RM Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.
- Kaestner L Theoretical Medicine and Biosciences, Saarland University, Saarbrücken, Germany.
- van Wijk R Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.
- 2018-06-19
Published in:
- Frontiers in physiology. - 2018
deformability
enzymopathies
hemolysis
hereditary spherocytosis
hydration
sickle cell anemia
thalassemia
vesiculation
English
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.
- Language
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- English
- Open access status
- gold
- Identifiers
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- DOI 10.3389/fphys.2018.00656
- PMID 29910743
- Persistent URL
- https://folia.unifr.ch/global/documents/4998
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