Abnormal parachloromercuriphenylsulfonate-sensitive cation channel in the erythrocytes of hereditary spherocytosis. 1976

T Kitao, and K Hattori, and M Takeshita

The erythrocytes of hereditary spherocytosis (HS) demonstrate an increased inward movement of sodium ions, an alteration which has been proposed as the primary defect leading to cell destruction. Parachloromercuriphenylsulfonate (PCMBS), an agent reacting with sulfhydryl groups of the membrane, increases the cation permeability of normal red cells, but does so to a much lesser extent in the HS red cells. On the other hand, pronase that is specific for amino groups of the membrane increases cation permeability and decreases anion permeability equally in normal and HS red cells. It may be postulated that a decreased number of sulfhydryl sites or a mutation of proteins in the PCMBS-sensitive cation channels of the HS cell membrane result in this hyposensitivity to PCMBS.

UI MeSH Term Description Entries
D009942 Organometallic Compounds A class of compounds of the type R-M, where a C atom is joined directly to any other element except H, C, N, O, F, Cl, Br, I, or At. (Grant & Hackh's Chemical Dictionary, 5th ed) Metallo-Organic Compound,Metallo-Organic Compounds,Metalloorganic Compound,Organometallic Compound,Metalloorganic Compounds,Compound, Metallo-Organic,Compound, Metalloorganic,Compound, Organometallic,Compounds, Metallo-Organic,Compounds, Metalloorganic,Compounds, Organometallic,Metallo Organic Compound,Metallo Organic Compounds
D011188 Potassium An element in the alkali group of metals with an atomic symbol K, atomic number 19, and atomic weight 39.10. It is the chief cation in the intracellular fluid of muscle and other cells. Potassium ion is a strong electrolyte that plays a significant role in the regulation of fluid volume and maintenance of the WATER-ELECTROLYTE BALANCE.
D002414 Cations, Monovalent Positively charged atoms, radicals or group of atoms with a valence of plus 1, which travel to the cathode or negative pole during electrolysis. Monovalent Cation,Cation, Monovalent,Monovalent Cations
D002463 Cell Membrane Permeability A quality of cell membranes which permits the passage of solvents and solutes into and out of cells. Permeability, Cell Membrane
D002731 4-Chloromercuribenzenesulfonate A cytotoxic sulfhydryl reagent that inhibits several subcellular metabolic systems and is used as a tool in cellular physiology. Chloromercuriphenylsulfonate,PCMBS,Chloromercuribenzene-p-sulphonic Acid,Chloromercuribenzenesulfonate,PCMPS,p-Chloromercuriphenylsulphonate,4 Chloromercuribenzenesulfonate,Acid, Chloromercuribenzene-p-sulphonic,Chloromercuribenzene p sulphonic Acid,p Chloromercuriphenylsulphonate
D004913 Erythrocytes, Abnormal Oxygen-carrying RED BLOOD CELLS in mammalian blood that are abnormal in structure or function. Abnormal Erythrocytes,Abnormal Erythrocyte,Erythrocyte, Abnormal
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012964 Sodium A member of the alkali group of metals. It has the atomic symbol Na, atomic number 11, and atomic weight 23. Sodium Ion Level,Sodium-23,Ion Level, Sodium,Level, Sodium Ion,Sodium 23
D013102 Spherocytes Small, abnormal spherical red blood cells with more than the normal amount of hemoglobin. Spherocyte
D013103 Spherocytosis, Hereditary A group of familial congenital hemolytic anemias characterized by numerous abnormally shaped erythrocytes which are generally spheroidal. The erythrocytes have increased osmotic fragility and are abnormally permeable to sodium ions. Hereditary Spherocytoses,Spherocytoses, Hereditary

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