Abnormal erythrocyte fragmentation and membrane deformability in paroxysmal nocturnal hemoglobinuria
Abnormal erythrocyte fragmentation and membrane deformability in paroxysmal nocturnal hemoglobinuria
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阵发性睡眠性血红蛋白尿症中红细胞破碎和膜变形异常
DOI：
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发表时间：
1985
期刊：
影响因子：
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通讯作者：
Brian D. Smith
中科院分区：
文献类型：
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作者：
Brian D. Smith
阵发性睡眠性血红蛋白尿症（PNH）的溶血被认为是固有膜缺陷的结果。这种缺陷可能导致PNH红细胞的异常材料特性。为了检验这一假设，在不存在补体的情况下，通过微量移液管技术评估了断裂失败和膜变形性。通过观察变形细胞的松弛来确定膜粘度。结果表明，双峰分布的膜故障，膜粘弹性，和弹性剪切模量的力。一个群体需要显著更小的破碎力，平均为0.56 × 10−6达因;膜粘度增加，平均为0.205 × 10−2达因秒/厘米;弹性剪切模量降低，平均为0.56 × 10−2达因/厘米。第二个群体与对照组相似，破碎力平均值为1.19 × 10− 6达因，对照组为1.05 × 10−6达因;膜粘度平均值为0.112 × 10 −2达因/厘米，对照组为0.102 × 10−2达因/厘米;弹性剪切模量平均值为0.70 × 10−2达因/厘米，对照组为0.78 × 10−2达因/厘米。具有异常材料性质的细胞的百分比对应于通过补体裂解测定的PNH III细胞的百分比。因此，溶血归因于异常克隆的红细胞在PNH是与内在膜异常，易于溶解。
Hemolysis in paroxysmal nocturnal hemoglobinuria (PNH) is considered to be a result of an intrinsic membrane defect. This defect may result in abnormal material properties of PNH erythrocytes. To examine this hypothesis, fragmentation failure, and membrane deformability were assessed in the absence of complement by micropipette techniques. Membrane viscosity was determined by observing relaxation of deformed cells. Results show a bimodal distribution of force for membrane failure, membrane viscoelasticity, and elastic shear modulus. One population requires significantly less force for fragmentation, mean 0.56 × 10−6 dyne; has increased membrane viscosity, mean 0.205 × 10−2 dyne sec/cm; and has decreased elastic shear modulus, mean 0.56 × 10−2 dyne/cm. A second population resembles control with fragmentation force, mean 1.19 × 10−6 dyne, control 1.05 × 10−6 dyne; membrane viscosity, mean 0.112 × 10−2 dyne/cm, control 0.102 × 10−2 dyne sec/cm; elastic shear modulus, mean 0.70 × 10−2 dyne/cm, control 0.78 × 10−2 dyne/cm. The percent of cells with abnormal material properties corresponds to the percent of PNH III cells determined by complement lysis. Thus, the hemolysis attributed to an abnormal clone of erythrocytes in PNH is associated with an intrinsic membrane abnormality which predisposes to lysis.
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DOI：
10.1073/pnas.80.17.5430
发表时间：
1983-01-01
期刊：
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES
影响因子：
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作者：
PANGBURN, MK;SCHREIBER, RD;MULLEREBERHARD, HJ
通讯作者：
MULLEREBERHARD, HJ
影响因子：
20.3
作者：
O. Linderkamp;H. Meiselman
通讯作者：
O. Linderkamp;H. Meiselman
影响因子：
20.3
作者：
G. Nash;C. Johnson;H. Meiselman
通讯作者：
G. Nash;C. Johnson;H. Meiselman
部分遗传性球形红细胞增多症家系红细胞膜骨架分子缺陷的鉴定。
影响因子：
20.3
作者：
Goodman,SR;Shiffer,KA;Casoria,LA;Eyster,ME
通讯作者：
Eyster,ME
DOI：
10.1073/pnas.80.16.5066
发表时间：
1983-01-01
期刊：
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES
影响因子：
--
作者：
NICHOLSONWELLER, A;MARCH, JP;AUSTEN, KF
通讯作者：
AUSTEN, KF