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Evidence for Imbalanced Furosemide‐Sensitive Na + , K + Cotransport in Hereditary Stomatocytosis
Author(s) -
Chailley Bernadette,
Feo Claude,
Garay Ricardo,
Dagher Georges,
Bruckdorfer Richard,
Fischer Siegmund,
Piau JeanPierre,
Delaunay Jean
Publication year - 1981
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1981.tb00498.x
Subject(s) - cotransporter , chemistry , bumetanide , ouabain , red blood cell , permeability (electromagnetism) , microviscosity , membrane , biophysics , sodium , intracellular , microbiology and biotechnology , biochemistry , biology , organic chemistry
The red cells from 5 related patients with hereditary stomatocytosis were investigated. Maximal rate constant of Na + passive permeability was increased while that of K + passive permeability was nearly normal. Ouabain‐sensitive Na + efflux was elevated. The Na + component of furosemide‐sensitive Na + , K + cotransport was also increased. However, its K + component, determined in 2 patients, remained within normal limits, thus departing from the strict 1:1 stoichiometry of the Na + , K + cotransport system. Yet, intracellular Na + and K + concentrations displayed limited and inconstant changes. A variety of abnormally‐shaped cells, including stomatocytes, were observed in scanning electron micrographs. Upon differential centrifugation, reticulocytes usually concentrated in the most dense region of the gradient. Red cell deformability, as studied by ektacytometry, was reduced. Membrane phosphatidylcholines and sphingomyelins were increased and decreased, respectively, whereas fatty acid distribution was unchanged. Membrane microviscosity was normal.