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产品规格
售价
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BN40577R-100ul
100ul
¥2360.00
交叉反应：Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit,Sheep) 推荐应用：IHC-P,IHC-F,ICC,IF,ELISA
BN40577R-200ul
200ul
¥3490.00
交叉反应：Mouse,Rat(predicted:Human,Dog,Pig,Cow,Horse,Rabbit,Sheep) 推荐应用：IHC-P,IHC-F,ICC,IF,ELISA
产品描述
| 英文名称 | SMCHD1 |
| 中文名称 | SMCHD1蛋白抗体 |
| 别 名 | KIAA0650; SMC hinge domain containing 1; SMC hinge domain containing protein 1; Smchd1; SMHD1_HUMAN; Structural maintenance of chromosomes flexible hinge domain containing 1; Structural maintenance of chromosomes flexible hinge domain-containing protein 1. |
| 研究领域 | 细胞生物 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Mouse, Rat, (predicted: Human, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) |
| 产品应用 | ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 226kDa |
| 细胞定位 | 细胞核 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SMCHD1:761-860/2005 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 产品介绍 | This gene encodes a protein which contains a hinge region domain found in members of the SMC (structural maintenance of chromosomes) protein family. [provided by RefSeq, Dec 2011] Function: Required for maintenance of X inactivation in females and hypermethylation of CpG islands associated with inactive X. Involved in a pathway that mediates the methylation of a subset of CpG islands slowly and requires the de novo methyltransferase DNMT3B (By similarity). Required for DUX4 silencing in somatic cells. Subcellular Location: Chromosome DISEASE: The disease is caused by mutations affecting the gene represented in this entry. SMCHD1 mutations lead to DUX4 expression in somatic tissues, including muscle cells, when an haplotype on chromosome 4 is permissive for DUX4 expression. Ectopic expression of DUX4 in skeletal muscle activates the expression of stem cell and germline genes, and, when overexpressed in somatic cells, DUX4 can ultimately lead to cell death. Disease description:A degenerative muscle disease characterized by slowly progressive weakness of the muscles of the face, upper-arm, and shoulder girdle. The onset of symptoms usually occurs in the first or second decade of life. Affected individuals usually present with impairment of upper extremity elevation. This tends to be followed by facial weakness, primarily involving the orbicularis oris and orbicularis oculi muscles. SWISS: A6NHR9 Gene ID: 23347 Database links: Entrez Gene: 23347 Human Entrez Gene: 74355 Mouse Omim: 614982 Human SwissProt: A6NHR9 Human SwissProt: Q6P5D8 Mouse Unigene: 8118 Human Unigene: 194450 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
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