| 產(chǎn)品編號 | bs-4177R-BF350 |
| 英文名稱(chēng) | Rabbit Anti-SOX9/BF350 Conjugated antibody |
| 中文名稱(chēng) | BF350標記的轉錄因子SOX9蛋白抗體 |
| 別 名 | CMD 1; CMD1; CMPD 1; CMPD1; SOX 9; Sox9; SOX9_HUMAN; SRA 1; SRA1 antibody SRY (sex determining region Y) box 9 (campomelic dysplasia autosomal; SRY (sex determining region Y) box 9; SRY (sex determining region Y)-box 9; SRY (sex-determining region Y)-box 9 protein; Transcription factor SOX 9; Transcription factor SOX-9; transcription factor SOX9; campomelic dysplasia autosomal sex reversal. |
| 規格價(jià)格 | 100ul/2980元 購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 腫瘤 細胞生物 免疫學(xué) 發(fā)育生物學(xué) 染色質(zhì)和核信號 神經(jīng)生物學(xué) 轉錄調節因子 表觀(guān)遺傳學(xué) |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | Human, (predicted: Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, ) |
| 產(chǎn)品應用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 56kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SOX9 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 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: The protein encoded by this gene recognizes the sequence CCTTGAG along with other members of the HMG-box class DNA-binding proteins. It acts during chondrocyte differentiation and, with steroidogenic factor 1, regulates transcription of the anti-Muellerian hormone (AMH) gene. Deficiencies lead to the skeletal malformation syndrome campomelic dysplasia, frequently with sex reversal. [provided by RefSeq]. Function: Plays an important role in the normal skeletal development. May regulate the expression of other genes involved in chondrogenesis by acting as a transcription factor for these genes. Subcellular Location: Nucleus (Potential). DISEASE: Defects in SOX9 are the cause of campomelic dysplasia (CMD1) [MIM:114290]. CMD1 is a rare, often lethal, dominantly inherited, congenital osteochondrodysplasia, associated with male-to-female autosomal sex reversal in two-thirds of the affected karyotypic males. A disease of the newborn characterized by congenital bowing and angulation of long bones, unusually small scapulae, deformed pelvis and spine and a missing pair of ribs. Craniofacial defects such as cleft palate, micrognatia, flat face and hypertelorism are common. Various defects of the ear are often evident, affecting the cochlea, malleus incus, stapes and tympanum. Most patients die soon after birth due to respiratory distress which has been attributed to hypoplasia of the tracheobronchial cartilage and small thoracic cage. Defects in SOX9 are the cause of 46,XX sex reversal type 2 (SRXX2) [MIM:278850]. SRXX2 is a condition in which male gonads develop in a genetic female (female to male sex reversal). Similarity: Contains 1 HMG box DNA-binding domain. Database links: Entrez Gene: 6662 Human Entrez Gene: 20682 Mouse Omim: 608160 Human SwissProt: P48436 Human SwissProt: Q04887 Mouse Unigene: 647409 Human Unigene: 286407 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. ????Sox9是軟骨形成過(guò)程中一個(gè)十分關(guān)鍵的轉錄因子，已經(jīng)有研究表明骨形成蛋白2(bone morphogenetic protein 2,BMP2)能誘導Sox9的表達。 ????有學(xué)者認為；Sox9蛋白很可能與椎間盤(pán)退變有著(zhù)密切的關(guān)系，Sox9是膠原蛋白合成過(guò)程中的一個(gè)重要的轉錄因子，且在軟骨的發(fā)育、成熟過(guò)程中對膠原蛋白有著(zhù)正向調控作用，Sox9蛋白在男性性腺調節睪丸發(fā)育中也有一定的作用。 |
