Survival and Motor Phenotypes in FVB C9-500 ALS/FTD BAC Transgenic Mice Reproduced by Multiple Labs

Lien Nguyen; Laboissonniere Lauren A.; Guo Shu; Pilotto Federica; Scheidegger Olivier; Oestmann Angelina; Hammond Jennetta W.; Li Herman; Hyysalo Anu; Peltola Roosa; Pattamatta Amrutha; Zu Tao; Voutilainen Merja H.; Gelbard Harris A.; Saxena Smita; Ranum Laura P. W.

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Survival and Motor Phenotypes in FVB C9-500 ALS/FTD BAC Transgenic Mice Reproduced by Multiple Labs

机译：多个实验室再现的FVB C9-500 ALS / FTD BAC转基因小鼠的存活和运动表型

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Mordes et al. (2020) did not detect the survival or motor phenotypes in C9orf72 BAC transgenic mice originally described by Liu et al. (2016) . We discuss methodological differences between the Mordes and Liu studies, several additional studies in which survival and motor phenotypes were found, and possible environmental and genetic effects. First, Nguyen et al. (2020) showed robust ALS/FTD phenotypes in C9-BAC versus non-transgenic (NT) mice and that alpha-GA(1) treatment improved survival, behavior, and neurodegeneration. The groups of Gelbard and Saxena also show decreased survival of C9-BAC versus NT mice and neuropathological and behavioral deficits similar to those shown by Liu et al. (2016) . Although FVB/N mice can have seizures, increases in seizure severity and death of C9 and NT animals, which may mask C9 disease phenotypes, have been observed in recent C9-500 FVB/NJ-bred cohorts. In summary, we provide an update on phenotypes seen in FVB C9-BAC mice and additional details to successfully use this model. This Matters Arising Response paper addresses the Mordes et al. (2020) Matters Arising paper, published concurrently in Neuron.

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《Neuron》 |2020年第4期|共16页
作者
Lien Nguyen; Laboissonniere Lauren A.; Guo Shu; Pilotto Federica; Scheidegger Olivier; Oestmann Angelina; Hammond Jennetta W.; Li Herman; Hyysalo Anu; Peltola Roosa; Pattamatta Amrutha; Zu Tao; Voutilainen Merja H.; Gelbard Harris A.; Saxena Smita; Ranum Laura P. W.;
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Univ Florida Coll Med Dept Mol Genet &

Microbiol Ctr NeuroGenet Genet Inst Gainesville FL;

Univ Florida Coll Med Dept Mol Genet &

Microbiol Ctr NeuroGenet Genet Inst Gainesville FL;

Univ Florida Coll Med Dept Mol Genet &

Microbiol Ctr NeuroGenet Genet Inst Gainesville FL;

Univ Hosp Bern Inselspital Dept Neurol CH-3010 Bern Switzerland;

Univ Hosp Bern Inselspital Dept Neurol CH-3010 Bern Switzerland;

Univ Hosp Bern Inselspital Dept Neurol CH-3010 Bern Switzerland;

Univ Rochester Ctr Neurotherapeut Discovery Dept Neurol Rochester NY 14618 USA;

Univ Rochester Ctr Neurotherapeut Discovery Dept Neurol Rochester NY 14618 USA;

Univ Helsinki Inst Biotechnol Helsinki 00790 Finland;

Univ Helsinki Inst Biotechnol Helsinki 00790 Finland;

Univ Florida Coll Med Dept Mol Genet &

Microbiol Ctr NeuroGenet Genet Inst Gainesville FL;

Univ Florida Coll Med Dept Mol Genet &

Microbiol Ctr NeuroGenet Genet Inst Gainesville FL;

Univ Helsinki Inst Biotechnol Helsinki 00790 Finland;

Univ Rochester Ctr Neurotherapeut Discovery Dept Neurol Rochester NY 14618 USA;

Univ Hosp Bern Inselspital Dept Neurol CH-3010 Bern Switzerland;

Univ Florida Coll Med Dept Mol Genet &

Microbiol Ctr NeuroGenet Genet Inst Gainesville FL;

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正文语种 eng
中图分类神经病学;
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1. Synaptophysin expression in motor neurons of transgenic mice with amyotrophic lateral sclerosis [J] . Juan Liu, Dawei Zang, Surindar Cheema 中国神经再生研究（英文版） . 2006,第008期
2. Downregulated miR-18b-5p triggers apoptosis by inhibition of calcium signaling and neuronal cell differentiation in transgenic SOD1(G93A)mice and SOD1(G17S and G86S)ALS patients [J] . Ki Yoon Kim, Yu Ri Kim, Kyung Won Choi, 转化神经变性病(英文) . 2020,第003期
3. Modeling ALS/FTD in mice: Updates on the C9orf72 BAC transgenic mice [J] . Le Bras Alexandra Lab Animal: Information, Ideas, Methods and Materials for the Animal Research Professional . 2021,第1期

机译：在小鼠中建模ALS / FTD：C9ORF72 BAC转基因小鼠的更新
4. Repeat length increases disease penetrance and severity in C9orf72 ALS/FTD BAC transgenic mice [J] . Pattamatta Amrutha, Nguyen Lien, Olafson Hailey R., Human Molecular Genetics . 2020,第24期

机译：重复长度增加C9ORF72 ALS / FTD BAC转基因小鼠中的疾病渗透和严重程度
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机译：C9ORF72-ALS / FTD：转基因小鼠快来成为BAC
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机译：用突变SOD1基因早期降低生存信号蛋白和脊柱运动神经元中的脊髓运动神经元的DNA修复酶
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机译：研究生存运动神经元基因的mRNA前剪接，以建模脊髓性肌萎缩症疾病表型。
8. C9orf72 BAC transgenic mice display typical pathologic features of ALS/FTD [O] . Jacqueline G. ORourke, Laurent Bogdanik, A.K.M.G. Muhammad, -1

机译：C9orf72 BAC转基因小鼠表现出ALS / FTD的典型病理特征
9. C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD [O] . O’Rourke Jacqueline G., Bogdanik Laurent, Muhammad A.K.M.G., 2015

机译：C9orf72 BAC转基因小鼠显示ALS / FTD的典型病理特征

Survival and Motor Phenotypes in FVB C9-500 ALS/FTD BAC Transgenic Mice Reproduced by Multiple Labs

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