MICE LACKING THE CNTF RECEPTOR, UNLIKE MICE LACKING CNTF, EXHIBIT PROFOUND MOTOR-NEURON DEFICITS AT BIRTH
MICE LACKING THE CNTF RECEPTOR, UNLIKE MICE LACKING CNTF, EXHIBIT PROFOUND MOTOR-NEURON DEFICITS AT BIRTH
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DOI：
10.1016/0092-8674(95)90172-8
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发表时间：
1995-10-20
期刊：
影响因子：
64.5
通讯作者：
YANCOPOULOS, GD
中科院分区：
文献类型：
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作者：
DECHIARA, TM;VEJSADA, R;YANCOPOULOS, GD
睫状神经营养因子 (CNTF) 在体外和运动神经元变性小鼠模型中支持运动神经元存活，并被认为是调节发育过程中运动神经元存活的肌肉源性神经营养活性的候选者。然而，CNTF在胚胎中的表达非常低，小鼠或人类中的CNTF基因突变不会导致发育中的神经系统出现显着异常。我们已经生成并直接比较了编码 CNTF 或其受体（CNTFR α）的基因中含有无效突变的小鼠。与缺乏 CNTF 的小鼠不同，缺乏 CNTFR α 的小鼠会在围产期死亡并表现出严重的运动神经元缺陷。因此，CNTFR α 对于发育中的神经系统至关重要，很可能是作为第二种对发育重要的 CNTF 样配体的受体。
Ciliary neurotrophic factor (CNTF) supports motor neuron survival in vitro and in mouse models of motor neuron degeneration and was considered a candidate for the muscle-derived neurotrophic activity that regulates motor neuron survival during development. However, CNTF expression is very low in the embryo, and CNTF gene mutations in mice or human do not result in notable abnormalities of the developing nervous system. We have generated and directly compared mice containing null mutations in the genes encoding CNTF or its receptor (CNTFR alpha). Unlike mice lacking CNTF, mice lacking CNTFR alpha die perinatally and display severe motor neuron deficits. Thus, CNTFR alpha is critical for the developing nervous system, most likely by serving as a receptor for a second, developmentally important, CNTF-like ligand.