Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase
Protective effect of neurofilament heavy gene overexpression in motor neuron disease induced by mutant superoxide dismutase
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DOI：
10.1073/pnas.95.16.9626
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发表时间：
1998-08-04
影响因子：
11.1
通讯作者：
Julien, JP
中科院分区：
文献类型：
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作者：
Couillard-Després, S;Zhu, QZ;Julien, JP
为了研究神经丝在超氧化物歧化酶（SOD 1）突变引起的运动神经元疾病中的作用，将表达肌萎缩侧索硬化症相关的SOD 1突变体（SOD 1（G37 R））的转基因小鼠与表达人神经丝重（NF-H）亚基的转基因小鼠交配。出乎意料的是，人NF-H转基因的表达增加了高达65%，即SOD 1（G37 R）小鼠的平均寿命。显微镜检查证实了NF-H蛋白对SOD 1毒性的保护作用。虽然大规模的神经变性发生在1岁大的小鼠表达SOD 1（G37 R）单独，脊髓根轴突和运动神经元显着幸免于双SOD 1（G37 R）; NF-H-转基因同窝出生。
To investigate the role of neurofilaments in motor neuron disease caused by superoxide dismutase (SOD1) mutations, transgenic mice expressing a amyotrophic lateral sclerosis-linked SOD1 mutant (SOD1(G37R)) were mated with transgenic mice expressing human neurofilament heavy (NF-H) subunits, Unexpectedly, expression of human NF-H transgenes increased by up to 65%, the mean lifespan of SOD1(G37R) mice. Microscopic examination corroborated the protective effect of NF-H protein against SOD1 toxicity. Although massive neurodegeneration occurred in 1-yr-old mice expressing SOD1(G37R) alone, spinal root axons and motor neurons were remarkably spared in doubly SOD1(G37R);NF-H-transgenic littermates.