FITC标记的双同源框蛋白4抗体
产品名称： FITC标记的双同源框蛋白4抗体
英文名称： Anti-DUX4/FITC
产品编号： HZ-12369R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-DUX4/FITC Conjugated antibody
FITC标记的双同源框蛋白4抗体
| 英文名称 | Anti-DUX4/FITC |
| 中文名称 | FITC标记的双同源框蛋白4抗体 |
| 别 名 | Double homeobox protein 10; Double homeobox protein 4; Double homeobox protein 4/10; DUX10; DUX4_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 发育生物学 信号转导 干细胞 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 45kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human DUX4 |
| 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 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: DUX4 is a homeodomain protein with a similar protein sequence to Pax3 and Pax7. Defects in DUX4 may be the cause of facioscapulohumeral muscular dystrophy (FSHD). FSHD is characterized by weakness of the muscles of the face, upper-arm and shoulder girdle. Severity is highly variable. Weakness is slowly progressive and about 20% of affected individuals eventually require a wheelchair. Approximately 70-90% of individuals have inherited the disease-causing deletion from a parent, and approximately 10-30% of affected individuals have FSHD as the result of a de novo deletion. Offsprings of an affected individual have a 50% chance of inheriting the deletion. Function: May be involved in transcriptional regulation. Subunit: May exist as a monomer or a dimer. Subcellular Location: Nucleus. Note=Actively transported through the nuclear pore complex (NPC). Tissue Specificity: Does not seem to be expressed in normal muscle, but in muscle of individuals with FSHD, where it may be toxic to cells. DISEASE: Defects in DUX4 may be the cause of facioscapulohumeral muscular dystrophy (FSHD) [MIM:158900]. FSHD is characterized by weakness of the muscles of the face, upper-arm and shoulder girdle. Severity is highly variable. Weakness is slowly progressive and about 20% of affected individuals eventually require a wheelchair. Approximately 70-90% of individuals have inherited the disease-causing deletion from a parent, and approximately 10-30% of affected individuals have FSHD as the result of a de novo deletion. Offsprings of an affected individual have a 50% chance of inheriting the deletion. Similarity: Belongs to the paired homeobox family. Contains 2 homeobox DNA-binding domains. Database links: UniProtKB/Swiss-Prot: Q9UBX2.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
Dux4是一种同源结构域蛋白，具有与PAX3和PAX7相似的蛋白序列。DUX4中的缺陷可能是面肩肱肌营养不良（FSHD）的原因。FSHD的特征是面部肌肉、上臂和肩胛带的肌肉无力。严重性是高度可变的。虚弱是缓慢渐进的，大约20%的受影响个体最终需要轮椅。大约70-90%的个体从父母那里遗传了引起疾病的缺失，大约10-30%的受影响个体由于新缺失而患有FSHD。受影响个体的后代有50%的机会继承删除。