Dellantonio R, Angeleri F, Capriotti M, Lenaz G, Curatola G, Mazzanti L, Bertoli E
Ital J Biochem. 1980 Mar-Apr;29(2):121-8.
Lipid-soluble spin labels were used to probe the fluidity of membrane lipids in erythrocyte membranes from patients with Duchenne muscular dystrophy. A greater mobility of spin label motion was noticed at the surface of the Duchenne erythrocyte membranes in comparison with the similary labeled normal human erythrocytes. Changes in the protein conformation and/or protein-lipid organization, as a consequence of an altered membrane lipid fluidity, have been demonstrated by the use of a sulfhydryl group specific protein spin label. Strongly immobilized sulfhydryl groups appear to be located both at the membrane surface and deep within the lipid bilayer. The ratio of the spectral amplitude of the spin label attached to weakly immobilized sulfhydryl groups to that of strongly immobilized SH-groups is significantly greater in Duchenne membranes, compared to those of controls. These studies suggest alterations in lipid-protein organization at the surface of Duchenne erythrocytes, indicating that Duchenne muscular dystrophy may be a disease resulting from a membrane abnormality at the molecular level.
脂溶性自旋标记物被用于探测杜兴氏肌营养不良症患者红细胞膜中膜脂的流动性。与同样标记的正常人类红细胞相比，在杜兴氏红细胞膜表面观察到自旋标记物运动的流动性更大。通过使用巯基特异性蛋白质自旋标记物，已证明由于膜脂流动性改变导致蛋白质构象和/或蛋白质-脂质组织发生变化。强烈固定的巯基似乎位于膜表面和脂质双层内部深处。与对照组相比，杜兴氏细胞膜中附着在弱固定巯基上的自旋标记物的光谱幅度与强固定巯基的光谱幅度之比明显更大。这些研究表明杜兴氏红细胞表面脂质-蛋白质组织发生了改变，表明杜兴氏肌营养不良症可能是一种由分子水平的膜异常引起的疾病。
