Abe K, Arashima S, Honma M
J Clin Pathol. 1982 May;35(5):496-501. doi: 10.1136/jcp.35.5.496.
Cultured fibroblasts from a patient with the Chediak-Higashi syndrome, the mother of the patient, and a normal control were studied by light and electron microscopy. The distribution pattern of PAS-positive and acid phosphatase-containing granules in the cytoplasm differed significantly in the fibroblasts from the patient when compared with those from the mother and control. The granules in the fibroblasts from the patient were clustered in the perinuclear area, whereas the granules in the fibroblasts from the mother and control were dispersed throughout the cytoplasm. After incubation with ascorbic acid, the clustered granules in the fibroblasts of the Chediak-Higashi syndrome showed a tendency to spread throughout the cytoplasm. The distribution pattern of the granules was studied by quantitative morphology.
对一名患有切-东综合征患者、该患者的母亲以及一名正常对照者的培养成纤维细胞进行了光镜和电镜研究。与患者母亲和对照者的成纤维细胞相比，患者成纤维细胞胞质中PAS阳性和含酸性磷酸酶颗粒的分布模式存在显著差异。患者成纤维细胞中的颗粒聚集在核周区域，而患者母亲和对照者成纤维细胞中的颗粒则分散于整个胞质中。用抗坏血酸孵育后，切-东综合征患者成纤维细胞中聚集的颗粒呈现出向整个胞质扩散的趋势。通过定量形态学研究了颗粒的分布模式。
