Abnormal parachloromercuriphenylsulfonate-sensitive cation channel in the
erythrocytes of hereditary spherocytosis
T Kitao, 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.
Volume 47,
Issue 4,
pp. 651-655,
04/01/1976
Copyright © 1976 by The American Society of Hematology
