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    Rabbit Anti-SOX9/RBITC Conjugated antibody (bs-10725R-RBITC)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-10725R-RBITC
    英文名稱(chēng) Rabbit Anti-SOX9/RBITC Conjugated antibody
    中文名稱(chēng) 羅丹明(RBITC)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, Pig, Horse, Rabbit, )
    產(chǎn)品應用 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 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

    Entrez Gene: 140586 Rat

    Omim: 608160 Human

    SwissProt: P48436 Human

    SwissProt: Q04887 Mouse

    SwissProt: O18896 Pig

    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.
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