Tissue culture loading test with storage granules from animal models of neuronal ceroid-lipofuscinosis (Batten disease): testing their lysosomal degradability by normal and Batten cells.
Tissue culture loading test with storage granules from animal models of neuronal ceroid-lipofuscinosis (Batten disease): testing their lysosomal degradability by normal and Batten cells.
复制标题
使用来自神经元蜡样质脂褐素沉着症（巴顿病）动物模型的储存颗粒进行组织培养负载试验：通过正常细胞和巴顿细胞测试其溶酶体降解性。
DOI：
10.1002/ajmg.1320570220
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发表时间：
1995
期刊：
影响因子：
--
通讯作者：
Palmer,DN
中科院分区：
文献类型：
--
作者：
Elleder,M;Drahota,Z;Lisa,V;Mares,V;Mandys,V;Muller,J;Palmer,DN
Storage granules (SGs) from ovine and canine models of Batten disease were found to be easily phagocytosed by four cell types studied. The cell types tested were human fibroblasts and peripheral monocytes (control and from a late infantile Batten disease patient), rat C6 cell line, and neonatal cardiomyocytes. The phagocytosed SGs elicited an increase in acid phosphatase activity which was localized in the phagolysosome. After phagocytosis SGs were followed for various times ranging from 7 to 21 days and were found to be of unchanged density (phase contrast), autofluorescence, and ultrastructural appearance. These findings point to their undegradability, or very low degree of degradability, in phagolysosomes in both normal or Batten cultured cells. The Batten disease SGs are not toxic and did not cause any adverse affect on the host cells. Either the normal clearance rate from lysosomes is too slow to be measured by this technique or subunit c accumulation in lysosomes need not result from a primary lysosomal protease defect. Subunit c may aggregate, because of the lack of some normally preventive factor, resulting in a physical barrier to the degradation of this highly apolar molecule. © 1995 Wiley‐Liss, Inc.
