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货号： bs-4177R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-4177R-Bio
- 英文名称
- Anti-SOX9/Biotin
- 中文名称
- 生物素标记的转录因子SOX9蛋白抗体
- 别 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 免疫学 发育生物学 染色质和核信号 神经生物学 转录调节因子 表观遗传学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit,
- 产品应用
- WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=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 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human SOX9
- 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:
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.
Sox9是软骨形成过程中一个十分关键的转录因子，已经有研究表明骨形成蛋白2(bone morphogenetic protein 2,BMP2)能诱导Sox9的表达。
有学者认为；Sox9蛋白很可能与椎间盘退变有着密切的关系，Sox9是胶原蛋白合成过程中的一个重要的转录因子，且在软骨的发育、成熟过程中对胶原蛋白有着正向调控作用，Sox9蛋白在男性性腺调节睾丸发育中也有一定的作用。