Journal
CURRENT OPINION IN HEMATOLOGY
Volume 20, Issue 3, Pages 201-207Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MOH.0b013e32835f6870
Keywords
ABCB6; erythrocyte; PIEZO1; RhAG; xerocytosis
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Funding
- Doris Duke Foundation, NIH [HL65448, DK62039]
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Purpose of review Regulation of erythrocyte volume homeostasis is critical for survival of the erythrocyte. Inherited or acquired disorders that perturb this homeostasis jeopardize the erythrocyte, leading to its premature destruction. This report reviews recent insights into pathways that influence cellular water and solute homeostasis and cell volume. Recent findings The molecular and genetic bases of primary disorders of erythrocyte hydration are beginning to be revealed. Recent studies have implicated roles for a new protein PIEZO1, a long sought after mammalian mechanosensory protein; GLUT1, the glucose transporter; SLC4A1, the anion transporter; RhAG, the Rh-associated glycoprotein; and ABCB6, an ATP-binding cassette family member. Secondary disorders associated with perturbed cellular volume and volume regulation include sickle cell disease, thalassemia, and hereditary spherocytosis, in which dehydration contributes to disease pathology and clinical complications. Advances in understanding the mechanisms regulating erythrocyte solute and water content, particularly associated with mechanotransduction pathways, have revealed novel mechanisms controlling erythrocyte hydration. Understanding these processes may provide innovative strategies to maintain normal erythrocyte volume in disorders associated with primary or secondary cellular dehydration. Summary Understanding the mechanisms controlling erythrocyte volume regulation will serve as a paradigm for other cells and may reveal new therapeutic targets for disease prevention and treatment.
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