产品中心
当前位置：首页>产品中心Anti-EXT2
货号： bs-14675R 基本售价： 1380.0 元 规格： 100ul
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-14675R
- 英文名称
- EXT2
- 中文名称
- 多发性外生骨疣蛋白2抗体
- 别 名
- Exostoses (multiple) 2; Exostosin 2; Exostosin-2; EXT2; EXT2_HUMAN; Glucuronosyl N acetylglucosaminyl proteoglycan 4 alpha N acetylglucosaminyltransferase; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 2; N acetylglucosaminyl proteoglycan 4 beta glucuronosyltransferase; Putative tumor suppressor protein EXT2; SOTV.
- 规格价格
- 100ul/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 100ul 200ul
- 研究领域
- 肿瘤 细胞生物 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Sheep,
- 产品应用
- ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 82kDa
- 细胞定位
- 细胞浆
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human EXT2:431-530/718
- 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.
- PubMed
- PubMed
- 产品介绍
- background:
This gene encodes one of two glycosyltransferases involved in the chain elongation step of heparan sulfate biosynthesis. Mutations in this gene cause the type II form of multiple exostoses. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008]
Function:
Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor.
Subunit:
Forms a homo/hetero-oligomeric complex with EXT1. Interacts with GALNT5.
Subcellular Location:
Endoplasmic reticulum membrane. Golgi apparatus membrane. The EXT1/EXT2 complex is localized in the Golgi apparatus.
Tissue Specificity:
Ubiquitous.
DISEASE:
Defects in EXT2 are a cause of hereditary multiple exostoses type 2 (EXT2)
[MIM:133701]. EXT is a genetically heterogeneous bone disorder caused by genes segregating on human chromosomes 8, 11, and 19 and designated EXT1, EXT2 and EXT3 respectively. EXT is a dominantly inherited skeletal disorder primarily affecting endochondral bone during growth. The disease is characterized by formation of numerous cartilage-capped, benign bone tumors (osteocartilaginous exostoses or osteochondromas) that are often accompanied by skeletal deformities and short stature. In a small percentage of cases exostoses have exhibited malignant transformation resulting in an osteosarcoma or chondrosarcoma. Osteochondromas development can also occur as a sporadic event.Defects in EXT2 are the cause of Potocki-Shaffer syndrome (PSS) [MIM:601224]. It is a contiguous gene syndrome due to proximal deletion of chromosome 11p11.2, including EXT2 and ALX4.
Similarity:
Belongs to the glycosyltransferase 47 family.
SWISS:
Q93063
Gene ID:
2132
Database links:Entrez Gene: 2132Human
Entrez Gene: 281151Cow
Entrez Gene: 14043Mouse
Entrez Gene: 311215Rat
Omim: 608210Human
SwissProt: O77783Cow
SwissProt: Q93063Human
SwissProt: P70428Mouse
Unigene: 368404Human
Unigene: 4336Mouse
Unigene: 2878Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.