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货号： bs-21246R-AF594 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-21246R-AF594
- 英文名称
- Anti-SLC26A2/AF594
- 中文名称
- AF594标记的溶质载体转运蛋白家族26成员2抗体
- 别 名
- D5S1708; Diastrophic dysplasia protein; Diastrophic dysplasia sulfate transporter; DTD; DTDST; EDM 4; EDM4; MST 153; MST153; MSTP 157; MSTP157; Solute carrier family 26 (anion exchanger) member 2; Solute carrier family 26 (sulfate transporter) member 2; Solute carrier family 26 member 2; Sulfate anion transporter 1; Sulfate transporter.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 发育生物学 信号转导 转运蛋白
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse,
- 产品应用
- 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
- 82kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human SLC26A2
- 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 diastrophic dysplasia sulfate transporter is a transmembrane glycoprotein implicated in the pathogenesis of several human chondrodysplasias. It apparently is critical in cartilage for sulfation of proteoglycans and matrix organization. [provided by RefSeq, Jul 2008]
Function:
Sulfate transporter. May play a role in endochondral bone formation.
Subcellular Location:
Membrane; Multi-pass membrane protein.
Tissue Specificity:
Ubiquitously expressed.
DISEASE:
The disease is caused by mutations affecting the gene represented in this entry.
Disease description:An autosomal recessive disease characterized by osteochondrodysplasia with clinical features including dwarfism, spinal deformation, and specific joint abnormalities.The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A form of achondrogenesis type 1, a lethal form of chondrodysplasia characterized by deficient ossification in the lumbar vertebrae and absent ossification in the sacral, pubic and ischial bones and clinically by stillbirth or early death. In addition to severe micromelia, there is a disproportionately large cranium due to marked edema of soft tissues.The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A perinatal dysplasia characterized by shortening of the limbs, a dysmorphic syndrome and radiographic skeletal features. Patients are stillborn or die soon after birth.The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A generalized skeletal dysplasia associated with significant morbidity. Joint pain, joint deformity, waddling gait, and short stature are the main clinical signs and symptoms. Radiological examination of the skeleton shows delayed, irregular mineralization of the epiphyseal ossification centers and of the centers of the carpal and tarsal bones. Multiple epiphyseal dysplasia is broadly categorized into the more severe Fairbank and the milder Ribbing types. The Fairbank type is characterized by shortness of stature, short and stubby fingers, small epiphyses in several joints, including the knee, ankle, hand, and hip. The Ribbing type is confined predominantly to the hip joints and is characterized by hands that are normal and stature that is normal or near-normal. Multiple epiphyseal dysplasia type 4 is a recessively inherited form, characterized by early childhood-onset hip dysplasia and recurrent patella dislocation. Short stature is not frequent.
Similarity:
Belongs to the SLC26A/SulP transporter (TC 2.A.53) family.
Contains 1 STAS domain.
Database links:Entrez Gene: 742297Chimpanzee
Entrez Gene: 101150948Gorilla
Entrez Gene: 1836Human
Entrez Gene: 100461101Orangutan
Omim: 606718Human
SwissProt: P50443Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.