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B6SJL-Tg(SOD1*G93A)1Gur/J
品系货号：002726 ｜ 通用名称：SOD1-G93A
又名：B6SJL.SOD1-G93A
这些SOD1-G93A 转基因小鼠表达携带G93A突变的人源SOD1，可用于研究神经肌肉疾病，如肌萎缩侧索硬化症（ALS 或 Lou Gehrig病）。
我们的临床前药效检测服务能够提供专业的科学经验和一系列基于靶点和基于表型的指标测定（包括体内指标和终点指标），以满足脊髓性肌萎缩小鼠模型中灵活的研究设计与检测方法开发。查看完整服务平台。
我们的临床前药效检测服务能够提供专业的科学经验和一系列基于靶点和基于表型的指标测定（包括体内指标和终点指标），以满足脊髓性肌萎缩小鼠模型中灵活的研究设计与检测方法开发。查看完整服务平台。
品系特点
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转基因
品系捐赠者 Dr. Mark Gurney - Tetra Discovery Partners
品系详情
SOD1-G93A（也称为G93A-SOD1）转基因半合子的小鼠可存活可育，并可表达携带G93A突变的人源SOD1。此首建鼠品系（通常称为 G1H）据报道具有高转基因拷贝数。半合子小鼠表现出类似于人类肌萎缩侧索硬化(ALS)的表型；由于脊髓运动神经元丧失，可出现一个或多个肢体瘫痪。转基因小鼠的寿命缩短：50%小鼠的寿命为128.9 +/- 9.3天（与之对比，C57BL/6J遗传背景下50%寿命为157.1 +/- 9.1天）。与LPS诱导的小胶质细胞和活化的M1/M2巨噬细胞不同，由疾病进展活化的脊髓小胶质细胞未上调M1（神经毒性）表型或M2（保护性）表型偏好的基因。在表达SOD1G93A的活化小胶质细胞中，基因表达模式呈现了独特的ALS特异性特征。在维持活体种群时，杰克森实验室发现雄性小鼠具有攻击性。我们建议每笼饲养不超过4只雄鼠。这些SOD1-G93A（也称为 G93A-SOD1）转基因小鼠可用于研究神经肌肉疾病，包括肌萎缩侧索硬化症（ALS或Lou Gehrig病）。
此品系运输时应携带RapID耳标。深入了解 RapID耳标。
品系建立
SOD1-G93A（或G93A-SOD1）转基因携带突变型人源SOD1基因（携带93号密码子甘氨酸至丙氨酸的单个氨基酸替换），由其内源性人SOD1启动子调控。将此转基因注射至B6SJLF1小鼠的受精卵中，获得首建鼠。 B6SJL混合遗传背景的转基因小鼠被送至杰克森实验室。
需要注意的是，在命名中G93A的描述是指，含有153个氨基酸的SOD1蛋白，第93位甘氨酸突变为丙氨酸（Tu et al. 1996 PNAS 93:3155 [PMID:8610185]）。在Ensembl数据库中的记录是SOD1蛋白含有154个氨基酸，如果依据此，其突变是G94A。这个记录上的不一致，有可能是源于目前认为这个蛋白的N端甲硫氨酸会被切掉（see Barra et al. 1980 FEBS Lett 120:53 [PMID:7002610]），而在数据库中的基因组序列信息中，通常是假定N端甲硫氨酸仍然存在。
参考文献
精选参考文献
当使用该小鼠品系发表文献时，请引用原始参考文献，并在材料方法中提供该品系的品系货号：JAX stock #002726。
1996
Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.
Tu PH , et al.
1994
Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation [see comments] [published erratum appears in Science 1995 Jul 14;269(5221):149]
Gurney ME , et al.
健康报告 屏障设施级别描述 >
育种须知 更多繁育和饲养支持 >
此品系通过用半合子携带者（优选雄性）与B6SJLF1杂交系交配来维持。维持活体种群时，杰克森实验室发现雄鼠具有攻击性，因此，我们建议一个饲养箱中雄鼠数量不超过4只。繁育获得的被毛颜色预期为“野鼠色且腹部发白、黑色、白化、棕色伴粉红色眼睛”。
繁育策略
B6SJLF1 雌鼠 X 半合子雄鼠
销售和使用条款
002726
SOD1-G93A
神经生物学、肌萎缩侧索硬化症
参考文献|
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精选参考文献当使用该小鼠品系发表文献时，请引用原始参考文献，并在材料方法中提供该品系的品系货号：JAX stock #002726
1996Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.
Tu PH , et al.
1994Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation [see comments] [published erratum appears in Science 1995 Jul 14;269(5221):149]
Gurney ME , et al. -
其它参考文献
2004Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model.
Dupuis L , et al.
2004Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis.
Kabashi E , et al.
2004The superoxide dismutase1 (sod1) G93A mutation does not promote neuronal injury after focal brain ischemia and optic nerve transection in mice.
Kilic E , et al.
2004Up-regulation and altered distribution of lysyl oxidase in the central nervous system of mutant SOD1 transgenic mouse model of amyotrophic lateral sclerosis.
Li PA , et al.
2004The arachidonic acid 5-lipoxygenase inhibitor nordihydroguaiaretic acid inhibits tumor necrosis factor alpha activation of microglia and extends survival of G93A-SOD1 transgenic mice.
West M , et al.
2004Caspase-12 cleavage and increased oxidative stress during motoneuron degeneration in transgenic mouse model of ALS.
Wootz H , et al.
2003Increased expression of the glial glutamate transporter EAAT2 modulates excitotoxicity and delays the onset but not the outcome of ALS in mice.
Guo H , et al.
2003Dissociation between neurodegeneration and caspase-11-mediated activation of caspase-1 and caspase-3 in a mouse model of amyotrophic lateral sclerosis.
Kang SJ , et al.
2003Hypoxic induction of vascular endothelial growth factor is selectively impaired in mice carrying the mutant SOD1 gene.
Murakami T , et al.
2003Iron porphyrin treatment extends survival in a transgenic animal model of amyotrophic lateral sclerosis.
Wu AS , et al.
2002Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse model.
Cifuentes-Diaz C , et al.
2002Lumbar motoneuron fate in a mouse model of amyotrophic lateral sclerosis.
Hamson DK , et al.
2002Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease.
Lino MM , et al.
2002Differential expression of inflammation- and apoptosis-related genes in spinal cords of a mutant SOD1 transgenic mouse model of familial amyotrophic lateral sclerosis.
Yoshihara T , et al.
2001Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation.
Andreassen OA , et al.
2001Leukemia inhibitory factor by systemic administration rescues spinal motor neurons in the SOD1 G93A murine model of familial amyotrophic lateral sclerosis.
Azari MF , et al.
2001Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis.
Guegan C , et al.
2000The prostate apoptosis response-4 protein participates in motor neuron degeneration in amyotrophic lateral sclerosis.
Pedersen WA , et al.
1995Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis.
Chiu AY , et al.
