Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS
Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS
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DOI：
10.1016/j.molbrainres.2004.07.002
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发表时间：
2004-11-04
期刊：
影响因子：
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通讯作者：
Heiman-Patterson, TD
中科院分区：
文献类型：
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作者：
Alexander, GM;Erwin, KL;Heiman-Patterson, TD
表达SOD 1的G93A突变形式的多个拷贝的转基因小鼠发展出与肌萎缩侧索硬化症（ALS）患者相似的运动神经元病理学和临床症状。这些小鼠的表型取决于其基因组中转基因拷贝的数量。转基因拷贝数的变化，虽然罕见，但有时会发生在交配时，由于减数分裂期间的基因座内重组事件。本研究的目的是建立一种真实的时间定量PCR方法，以确定这些小鼠中转基因拷贝数的变化，并评估转基因拷贝数对ALS的G93 A SOD I小鼠模型表型的影响。(C)2004 Elsevier B.V.保留所有权利。
Transgenic mice expressing multiple copies of the G93A mutant form of SOD1 develop motor neuron pathology and clinical symptoms similar to those seen in patients with amyotrophic lateral sclerosis (ALS). The phenotype of these mice is dependent on the number of transgene copies in their genome. Changes in transgene copy number, although rare, can sometimes occur while mating due to intra locus recombination events during meiosis. The objective of this study was to develop a real time quantitative PCR method to determine changes in transgene copy number in these mice and to evaluate the effect of transgene copy number on the phenotype of the G93A SOD I mouse model of ALS. (C) 2004 Elsevier B.V. All rights reserved.