Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions

Pang-Hsien Tu; Pramod Raju; Kathryn A. Robinson

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Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions

机译：携带人类突变型超氧化物歧化酶转基因的转基因小鼠发展出类似于人类肌萎缩性侧索硬化病灶的神经元细胞骨架病理

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Mutations in the human Cu,Zn superoxide dismutase gene (SOD1) are found in 20/100 of kindreds with familial amyotrophic lateral sclerosis. Transgenic mice (line G1H) expressing a human SOD1 containing a mutation of Gly-93 - Ala (G93A) develop a motor neuron disease similar to familial amyotrophic lateral sclerosis, but transgenic mice (line N1029) expressing a wild-type human SOD1 transgene do not.

机译：人类铜，锌超氧化物歧化酶基因（SOD1）的突变发现于家族性肌萎缩性侧索硬化症的20/100亲属中。表达包含Gly-93-Ala突变的人SOD1的转基因小鼠（品系G1H）（G93A）会发展出类似于家族性肌萎缩性侧索硬化症的运动神经元疾病，但是表达野生型人SOD1转基因的转基因小鼠（品系N1029）却能做到不。

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《Proceedings of the National Academy of Sciences of the United States of America》 |1996年第7期|p.3155-3160|共6页
作者
Pang-Hsien Tu; Pramod Raju; Kathryn A. Robinson;
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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种 eng
中图分类自然科学总论;
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1. Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease. [J] . Nagai M, Aoki M, Miyoshi I, The Journal of Neuroscience: The Official Journal of the Society for Neuroscience . 2001,第23期

机译：表达具有肌萎缩性侧索硬化的人胞质铜锌超氧化物歧化酶转基因的大鼠：相关突变发展为运动神经元疾病。
2. Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease. [J] . Nagai M, Aoki M, Miyoshi I, The Journal of Neuroscience: The Official Journal of the Society for Neuroscience . 2001,第23期

机译：表达具有肌萎缩性侧索硬化的人胞质铜锌超氧化物歧化酶转基因的大鼠：相关突变发展为运动神经元疾病。
3. Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice. [J] . Jaarsma D, Teuling E, Haasdijk ED, The Journal of Neuroscience: The Official Journal of the Society for Neuroscience . 2008,第9期

机译：突变型超氧化物歧化酶的神经元特异性表达足以在转基因小鼠中诱导肌萎缩性侧索硬化。
4. Identification of Three Mutations in the Cu,Zn-Superoxide Dismutase (Cu,Zn-SOD) Gene with Familial Amyotrophic Lateral Sclerosis: Transduction of Human Cu,Zn-SOD into PC12 Cells by HIV-1 TAT Protein Basic Domain [C] . CHIH-MING CHOU, CHANG-JEN HUANG, CHWEN-MING SHIH, Asian Society for Mitochondrial Research and Medicine . 2005

机译：鉴定Cu，Zn-超氧化物歧化酶（Cu，Zn-SOD）基因的三种突变与家族性肌肌疏远硬化剂：HIV-1 TAT蛋白碱性结构域的转导，Zn-SOD进入PC12细胞
5. Characterization of Familial Amyotrophic Lateral Sclerosis Associated Mutants of the Human CopperZinc Superoxide Dismutase Monomer by Molecular Dynamics Simulations. [D] . Schmidlin, Thomas E. 2011

机译：人类铜锌超氧化物歧化酶单体的家族性肌萎缩性侧索硬化相关突变体的分子动力学模拟表征。
6. Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. [O] . P H Tu, P Raju, K A Robinson, 1996

机译：携带人类突变型超氧化物歧化酶转基因的转基因小鼠发展出类似于人类肌萎缩性侧索硬化病灶的神经元细胞骨架病理。
7. Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. [O] . Tu, P H, Raju, P, Robinson, K A, 1996

机译：携带人类突变型超氧化物歧化酶转基因的转基因小鼠发展出类似于人类肌萎缩性侧索硬化病灶的神经元细胞骨架病理。

Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions

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