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dc.contributor.authorAgre, P
dc.contributor.authorWaugh, R E
dc.date.accessioned2010-02-03T19:05:30Z
dc.date.available2010-02-03T19:05:30Z
dc.date.issued1988-01
dc.identifier.citationJ Clin Invest. 1988 January; 81(1): 133–141. doi: 10.1172/JCI113284en_US
dc.identifier.urihttp://jhir.library.jhu.edu/handle/1774.2/33788
dc.description.abstractHereditary spherocytosis is a common hemolytic anemia associated with deficiencies in spectrin, the principal structural protein of the erythrocyte membrane-skeleton. We have examined 20 different individuals from 10 spherocytosis kindreds and 2 elliptocytosis kindreds to determine the effects of different levels of spectrin deficiency on the viscoelastic properties of the erythrocyte membrane. Micropipettes were used to perform single-cell micromechanical measurements of approximately 1,000 individual cells to determine the membrane elastic shear modulus, the apparent membrane bending stiffness, and whole cell recovery time constant for the different cell populations. The membrane viscosity was calculated by the product of the shear modulus and the recovery time constant. Results show correlation between the fractional reduction in shear modulus and the fractional reduction in spectrin content (determined by spectrin radioimmunoassay) and spectrin density (determined by the ratios of spectrin to band 3 on electrophoresis gels) suggesting that membrane shear elasticity is directly proportional to the surface density of spectrin on the membrane (P less than 0.001). The apparent membrane bending stiffness is also reduced in proportion to the density of spectrin (P less than 0.001). The membrane viscosity is reduced relative to control (P less than 0.001), but the nature of the relationship between spectrin density and membrane viscosity is less clearly defined. These studies document striking relationships between partial deficiencies of erythrocyte spectrin and specific viscoelastic properties of the mutant membranes.en_US
dc.language.isoen_USen_US
dc.publisherAmerican Society for Clinical Investigationen_US
dc.subjectSpherocytosis, Hereditary/blooden_US
dc.subjectSpectrin/deficiencyen_US
dc.subjectErythrocyte Membrane/physiologyen_US
dc.subjectErythrocyte Deformabilityen_US
dc.titleReductions of erythrocyte membrane viscoelastic coefficients reflect spectrin deficiencies in hereditary spherocytosisen_US
dc.typeArticleen_US


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