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Luanne L. Peters, Hitesh K. Jindel, Babette Gwynn, Cathy Korsgren, Kathryn M. John, Samuel E. Lux, Narla Mohandas, Carl M. Cohen, Michael R. Cho, David E. Golan, Carlo Brugnara
J Clin Invest. 1999;
103(11):1527
doi:10.1172/JCI5766
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rotein 4.2 is a major component of the red blood cell (RBC) membrane skeleton. We used targeted mutagenesis in embryonic stem (ES) cells to elucidate protein 4.2 functions in vivo. Protein 4.2–null (4.2–/–) mice have mild hereditary spherocytosis (HS). Scanning electron microscopy and ektacytometry confirm loss of membrane surface in 4.2–/– RBCs. The membrane skeleton architecture is intact, and the spectrin and ankyrin content of 4.2–/– RBCs are normal. Band 3 and band 3–mediated anion transport are decreased. Protein 4.2–/– RBCs show altered cation content (increased K+/decreased Na+)resulting in dehydration. The passive Na+ permeability and the activities of the Na-K-2Cl and K-Cl cotransporters, the Na/H exchanger, and the Gardos channel in 4.2–/– RBCs are significantly increased. Protein 4.2–/– RBCs demonstrate an abnormal regulation of cation transport by cell volume. Cell shrinkage induces a greater activation of Na/H exchange and Na-K-2Cl cotransport in 4.2–/– RBCs compared with controls. The increased passive Na+ permeability of 4.2–/– RBCs is also dependent on cell shrinkage. We conclude that protein 4.2 is important in the maintenance of normal surface area in RBCs and for normal RBC cation transport.
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