DNA fragmentation and activation of c‐Jun in the cerebellum of mutant mice (weaver, Purkinje cell degeneration)
DNA fragmentation and activation of c‐Jun in the cerebellum of mutant mice (weaver, Purkinje cell degeneration)
复制标题
突变小鼠小脑中的 DNA 片段化和 c-Jun 激活（weaver，浦肯野细胞变性）
DOI：
10.1097/00001756-199509000-00014
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发表时间：
1995
期刊：
影响因子：
1.7
通讯作者：
M. Zimmermann
中科院分区：
文献类型：
--
作者：
F. Gillardon;J. Bäurle;U. Grüsser‐Cornehls;M. Zimmermann
韦弗和浦肯野细胞变性（fcd）是小鼠的常染色体隐性突变，其特征分别是几乎完全丧失小脑浦肯野神经元和颗粒细胞。发育性神经元死亡是通过激活凋亡途径发生的，在编织突变体变性颗粒细胞中观察到染色质凝结。在本研究中，我们利用末端转移酶和荧光素- dUTP原位末端标记，证明了pcd小鼠浦肯野细胞和织布突变体颗粒细胞在神经元变性期间的核DNA断裂。此外，候选细胞死亡效应基因c-jun的激活已被免疫组织化学检测到仅在受影响的细胞群中。标记的DNA片段和核c-Jun免疫反应性在野生型动物中几乎不存在。因此，基因决定的pcd和weaver突变小鼠的细胞死亡具有凋亡的特征，可能需要激活细胞死亡效应基因。
Weaver and Purkinje cell degeneration (fcd) are auto- somal recessive mutations in the mouse characterized by an almost complete loss of cerebellar Purkinje neurones and granule cells, respectively. Developmental neuronal death occurs by activation of an apoptotic pathway and chromatin condensation has been observed in degenerating granule cells of weaver mutants. In the present study we demonstrate nuclear DNA fragmentation in Purkinje cells of pcd mice and in granule cells of weaver mutants during the period of neuronal degeneration using in situ end labelling by terminal transferase and fluorescein- dUTP. Furthermore, activation of candidate cell death effector gene c-jun has been detected exclusively within the affected cell populations by immunohistochemistry. Both labelled DNA fragments and nuclear c-Jun immunoreactivity were virtually absent in wild-type animals. Thus, genetically determined cell death in pcd and weaver mutant mice has features of apoptosis and may require activation of cell death effector genes.