- 中文名称
SMN2 polyclonal antibody
- 英文名字
- SMN2 polyclonal antibody
- 供应商
- Bioworld
- 产品货号
- BS8018
- 产品报价
- ￥询价/50ul
- 产品说明书
- 点击查看
- 购买方式
- 银行转账、电汇、支票、现金，在线支付宝及网银支付，或直接与我们电话联系400-6800-868
- 产品新闻
- 背景资料
- Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations of SMN (survival of motor neuron) gene. SMN, also known as Gemin1, SMN1, SMNT and BCD541, exists as four isoforms produced by alternative splicing. SMN is oligomeric and forms a complex with Gemin2 (formerly SIP1), Gemin3 (a DEAD box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (gemini of the coiled bodies). Cytoplasmic SMN interacts with spliceosomal Sm proteins and facilitates their assembly onto U snRNAs, and nuclear SMN mediates recycling of pre-mRNA splicing factors. Nearly identical telomeric and centromeric forms of SMN encode the same protein; however, only mutations in the telomeric form are associated with the disease-state SMA. SMN is expresed in a wide variety of tissues including brain, kidney, liver, spinal cord and moderately in skeletal and cardiac muscle.
- 应用类型
- WB, IF/ICC, IP
- 免疫原
- Recombinant full length Human SMN2.
- 来源宿主
- Rabbit
- 反应性
- Human, Mouse, Rat
- 保存建议
- Store at 4
- 其他
- Bioworld是专一服务于生命科学研究的专业技术型企业，Bioworld品牌创立以来拥有10000多种常规产品现货，现有8000多种现货抗体，以及其他IVD原料，蛋白等现货产品。在细胞信号通路、免疫学、蛋白组学上拥有显著优势，其抗体产品、生长因子、IVD原料等为生命科学科研工作者提供了极大的便利
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该页面的中文产品信息的翻译，仅供参考。准确的产品信息请以厂家的英文说明书为准。下单前，请浏览说明书确认。
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