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Anti-SMN2 Antibody, clone SMN-KH
产品别名
Anti-SMN2 Antibody, clone SMN-KH
Survival motor neuron protein, Component of gems 1, Gemin-1
基本信息
| eCl@ss | 32160702 |
| NACRES | NA.41 |
| General description【一般描述】 | Survival of Motor Neuron (SMN) is expressed from two linked paralogous genes, SMN1 and SMN2. SMNs are primarily localized in the cytoplasm and nuclear gems of all cells, where they are understood to mediate the assembly of spliceosomal small nuclear ribonucleoprotein particles (snRNPs). They also play a role in the metabolism of snoRNPs and are required for the splicing of pre-mRNA in the nucleus. The progressive loss of functional SMNs in the anterior horn of the spinal cord is a critical cause of Spinal Muscular Atrophy (SMA), an autosomal recessive neuromuscular disease. There are three known types of childhood-onset SMA, in addition to a fourth type that is characterized by adult-onset SMA. |
| Immunogen【免疫原】 | MBP-tagged recombinant protein corresponding to human SMN2. |
| Application【应用】 | Anti-SMN2 Antibody, clone SMN-KH is a Mouse Monoclonal Antibody for detection of SMN2 also known as Survival motor neuron protein & has been validated in WB & IHC. Western Blot Analysis: A representative lot from an independent laboratory detected SMN2 in HeLa cell lysate. Immunohistochemistry Analysis: A respresentative lot from an independent laboratory detected human SMN2 in SMA type III mouse model thoracic spinal cord tissue. (Hua, Y., et al. (2010). Genes Dev. 24(15):1634-1644.) |
| Quality【质量】 | Evaluated by Western Blot in HeLa cell lysate. Western Blot Analysis: 0.5 µg/mL of this antibody detected SMN2 in 10 µg of HeLa cell lysate. |
| Physical form【外形】 | Protein G Purified Format: Purified |
| Other Notes【其他说明】 | Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration. |
产品性质
| Quality Level【质量水平】 | 100 |
| biological source【生物来源】 | mouse |
| antibody form【抗体形式】 | purified immunoglobulin |
| antibody product type | primary antibodies |
| clone【克隆】 | SMN-KH, monoclonal |
| species reactivity | human |
| technique(s) | immunohistochemistry: suitable western blot: suitable |
| isotype【同位素/亚型】 | IgG1κ |
| NCBI accession no.【NCBI登记号】 | NP_000335 |
| UniProt accession no.【UniProt登记号】 | Q16637 |
| shipped in【运输】 | wet ice |
| Gene Information | human ... SMN2(6607) |
产品说明
| Target description【目标描述】 | ~35 kDa observed |
| 【免责声明】 | Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. |
安全信息
| Storage Class Code【储存分类代码】 | 12 - Non Combustible Liquids |
| WGK | WGK 1 |
