| 產(chǎn)品編號(hào) | bs-12369R-BF488 |
| 英文名稱1 | Rabbit Anti-DUX4/BF488 Conjugated antibody |
| 中文名稱 | BF488標(biāo)記的雙同源框蛋白4抗體 |
| 別 名 | Double homeobox protein 10; Double homeobox protein 4; Double homeobox protein 4/10; DUX10; DUX4_HUMAN. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 發(fā)育生物學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 干細(xì)胞 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, ) |
| 產(chǎn)品應(yīng)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 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human DUX4 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
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. |
