Regular ArticleThe hereditary stomatocytoses and allied disorders: congenital disorders of erythrocyte membrane permeability to Na and K
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Cited by (41)
Disorders of erythrocyte hydration
2017, BloodCitation Excerpt :Primary disorders are classified by the perturbation of intracellular water content (ie, overhydration or dehydration), which reflects the abnormality in membrane permeability to sodium and potassium. Another classification system utilizes temperature dependence of the cation leak observed.4 Initially, these were called hereditary stomatocytosis syndromes because of the variable numbers of stomatocytes observed on peripheral blood smear from affected patients.
A hypothesis of target cell formation in sickle cell disease
2016, Medical HypothesesCitation Excerpt :However, the Gardos channel which decreases cell KCl concentration by its activation by Ca2+ also contributes to target cell formation since of an abnormal Ca2+ homeostasis in sickle cell disease [21,22] and that there is an inhibition of the decrease of cell KCl concentration in this disease by the antifungal agent clotrimazole, an effective inhibitor of this channel [23]. The endogenous Pi would derive from the faster 2,3-BPG catabolism leading to ATP formation by pyruvate kinase, the penultimate enzyme of the glycolytic pathway, an increase of ATP hydrolysis by the membrane Na+, K+-ATPase in response to the perturbation of the normal cell NaCl and KCl concentrations [24] and ATP hydrolysis by the highly active membrane Ca2+-Mg2+-ATPase which minimizes the Ca2+ activating Gardos channel activity by extruding Ca2+ ions coupled with uptakes of H+ ions, further decreasing cell pH [21]. Fig. 1 illustrates the process of target cell formation in sickle cell disease.
Non-electrolyte permeability of deoxygenated sickle cells compared
2008, Blood Cells, Molecules, and DiseasesStomatocytosis of Standard Schnauzers is not associated with stomatin deficiency
2007, Veterinary JournalEukaryotic and prokaryotic stomatins: The proteolytic link
2004, Blood Cells, Molecules, and DiseasesBlood Cells: A Practical Guide, SIXTH EDITION
2021, Blood Cells: A Practical Guide, Sixth Edition
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All correspondence to: G. W. Stewart. Tel: 0171 209 6193; Fax: 0171 209 6211; e-mail: [email protected]