Enlarged dysmorphic lysosomes in an established beige (C57BL/6J;bgJ(/bgJ)) mouse mutant fibroblast line: a reversible characteristic.
Enlarged dysmorphic lysosomes in an established beige (C57BL/6J;bgJ(/bgJ)) mouse mutant fibroblast line: a reversible characteristic.
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已建立的米色 (C57BL/6J;bgJ(/bgJ)) 小鼠突变成纤维细胞系中畸形溶酶体增大：可逆特征。
DOI：
10.1007/bf02723046
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发表时间：
1996
期刊：
影响因子：
--
通讯作者：
Lainwala,S
中科院分区：
文献类型：
--
作者：
Gow,JB;Lyerla,TA;Lainwala,S
The beige mouse mutant is considered an animal model for human Chediak-Higashi Syndrome (CHS) because of many shared pheno typic similarities such as partial albinism (24, 31), natural killer cell deficiency (20, 21), and platelet storage pool deficiency (2, 13). In both mutants, the presence of enlarged cellular organelles including lysosomes and melanosomes is responsible for most of the phenotypic manifestations. The presence of giant lysosomes in blood cells, for example, leads to decreased chemotatic and bactericidal activity against invading organisms (4, 7, 22). Giant melanosomes cause dilute pigmentation in both beige mice and in CHS (10). The enlarged lysosomes seen in beige and CHS cells are appar ently derived from fusion of smaller lysosomes and late endosomes (3). In developing leukocytes of the beige mouse, ultrastructural stud ies suggest a high rate of fusion in younger cells to form the giant lysosomes seen in older cells (5). Serial sections of CHS neutrophils examined ultrastructurally demonstrated that normal-sized lyso somes were commonly present and independent of the larger organ elles. Several of these were identified in various stages of fusion to form giant lysosomes (29). In addition, the fusion of normal-appear ing lysosomes into giant organelles has been observed directly by time-lapse video phase-contrast microscopy in beige mouse fibro blasts (30).We demonstrated that established beige mouse fibroblasts which are extensively passaged show an increase in proliferation rate and lose their characteristic enlarged lysosomes (9). We now show that it is possible to manipulate the growth rate of these late passage cells and recover the enlarged lysosomes characteristic of the beige mutant cellular phenotype in these spontaneously transformed fibroblasts. The perinuclear distribution of these organelles, however, remains a consistent feature of these cells at any passage number and under any culture conditions. In addition, late passage beige cells grown in low serum conditions to slow their rate and then returned to a standard serum concentration show again the late passage phenotype with small lysosomes.
