ITO CELLS IN LYSOSOMAL STORAGE DISORDERS - AN ULTRASTRUCTURAL-STUDY
ITO CELLS IN LYSOSOMAL STORAGE DISORDERS - AN ULTRASTRUCTURAL-STUDY
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DOI：
10.1007/bf02890291
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发表时间：
1984-01-01
期刊：
影响因子：
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通讯作者：
ELLEDER, M
中科院分区：
文献类型：
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作者：
ELLEDER, M
对各种溶酶体贮积病患者的一系列肝活检进行超微结构研究，以评估Ito细胞（IC）中溶酶体肥大和增生的程度。在以前的研究中，这被认为是不存在的或只是初步的。通过存在被限制膜包围的储存细胞体及其内容物的外观（与其他肝储存溶酶体中的内容物相同）来识别溶酶体储存。在鞘磷脂酶缺乏症（尼曼-匹克病A、B型）、沃尔曼病、GM 1神经节苷脂沉积症、粘多糖沉积症和多发性硫酸酯酶缺乏症中发现储存。在C型尼曼-匹克病中，除了具有显著肝硬化的病例外，几乎不存在。储存程度不同，并伴有生理脂肪含量（细胞质脂滴）下降。IC受影响的程度仅与研究标本中非特异性成纤维细胞的参与程度相关，因此似乎反映了成纤维细胞群体中的储存。
An ultrastructural study was performed in a series of liver biopsies from patients with various lysosomal storage diseases to evaluate the extent of lysosomal hypertrophy and hyperplasia in Ito cells (IC). In previous studies this was considered to be absent or only rudimentary. Lysosomal storage was recognized by the presence of storage cytosomes surrounded by limiting membranes and by the appearance of their content which was identical to that in other hepatic storage lysosomes. Storage was found in sphingomyelinase deficiency (Niemann-Pick disease types A, B), in Wolman''s disease, GM1 gangliosidosis, mucopolysaccharidosis and in multiple sulfatase deficiency. In type C Niemann-Pick disease it was virtually absent with the exception of cases with prominent hepatic symptomatology. Storage was of variable degree and was accompanied by a decrease in the phsyiological fat content (cytoplasmic lipid droplets). The degree to which IC were affected correlated only with the extent of which nonspecific fibroblasts were involved in the specimens studied and thus seems to reflect storage in the fibroblastic population.