Cardiac and neurological abnormalities in v-fps transgenic mice.
Cardiac and neurological abnormalities in v-fps transgenic mice.
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v-fps 转基因小鼠的心脏和神经系统异常。
DOI：
10.1073/pnas.86.15.5873
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发表时间：
1989
影响因子：
11.1
通讯作者：
T. Pawson
中科院分区：
文献类型：
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作者：
S. Yee;D. Mock;V. Maltby;M. Silver;J. Rossant;A. Bernstein;T. Pawson
广泛表达v-fps蛋白-酪氨酸激酶的转基因小鼠除了肿瘤发病率高外，还会出现几种独立的病理情况。心脏中V-fps的表达和蛋白酪氨酸激酶活性与心脏增大直接相关。这种心脏增大伴有严重的心肌和心内膜损害，集中在左室壁，其特征是心肌纤维进行性萎缩和坏死，并伴有纤维化。这种病理与充血性心力衰竭有关。来自五个品系的小鼠出现了明显的颤抖，这与v-fps转基因在大脑中的表达有关，其中两个品系的小鼠三叉神经出现了显著的双侧扩大。与肿瘤形成不同，这些心脏和神经表型在出生后不久就很明显，并显示出100%的外显性。V-fps转基因的多效性表明蛋白酪氨酸激酶参与了哺乳动物的神经发育和心脏功能。
Transgenic mice that widely express the v-fps protein-tyrosine kinase develop several independent pathological conditions, in addition to a high tumor incidence. v-fps expression and protein-tyrosine kinase activity in the heart were directly correlated with cardiac enlargement. This cardiomegaly was accompanied by severe myocardial and endocardial damage, which was concentrated in the left ventricular wall, and characterized by a progressive atrophy and necrosis of cardiac muscle fibers with concomitant fibrosis. This pathology was associated with congestive heart failure. Mice from five lines developed a marked trembling, correlated with expression of the v-fps transgene in the brain, and two lines showed a striking bilateral enlargement of the trigeminal nerves. Unlike tumor formation, these cardiac and neurological phenotypes were evident shortly after birth and showed 100% penetrance. The pleiotropic effects of the v-fps transgene suggest the involvement of protein-tyrosine kinases in mammalian neural development and cardiac function.
《1985年度研究成果报告》（1986年）
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