Ostlund R E, Tucker R W, Leung J T, Okun N, Williamson J R
Blood. 1980 Nov;56(5):806-11.
The Chediak-Higashi syndrome (CHS) trait is expressed in cultured human skin fibroblasts as an abnormal perinuclear concentration of moderately enlarged lysosomes. The cytoskeleton of CHS fibroblasts appears intact. Microtubules are normal in number and morphology, as assessed by colchicine binding studies, antitubulin immunofluorescence, and electron microscopy. Deformability by shear force is unaltered and microfilaments are abundant. However, CHS lysosomes appear to interact abnormally with the cytoskeleton, since the perinculear aggregation partially disperses after depolymerization of cell microtubules with colchicine. These results suggest that CHS is associated with a defect of either the lysosomal membrane itself or of lysosomal membrane-microtubule interaction.
切-东综合征（CHS）特征在培养的人皮肤成纤维细胞中表现为中等大小的溶酶体在核周异常聚集。CHS成纤维细胞的细胞骨架看起来完整。通过秋水仙碱结合研究、抗微管蛋白免疫荧光和电子显微镜评估，微管数量和形态正常。剪切力引起的变形能力未改变，微丝丰富。然而，CHS溶酶体似乎与细胞骨架存在异常相互作用，因为用秋水仙碱使细胞微管解聚后，核周聚集部分分散。这些结果表明，CHS与溶酶体膜本身或溶酶体膜-微管相互作用的缺陷有关。
