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货号： bs-8296R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-8296R-AF555
- 英文名称
- Anti-Matriptase 2/AF555
- 中文名称
- AF555标记的膜型丝氨酸蛋白酶2抗体
- 别 名
- Matriptase-2; Matriptase2; Membrane type serine proteinase 2; MTSP 2; MTSP2; PVAE354; TMPRSS 6; TMPRSS6; TMPS6_HUMAN; TMSP 6; TMSP6; Transmembrane protease serine 6; Type II Membrane Serine Proteinase 6.
- 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 免疫学 细胞骨架
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Cow,
- 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
- 89kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Matriptase 2
- 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:
Serine protease which hydrolyzes a range of proteins including type I collagen, fibronectin and fibrinogen. Can also activate urokinase-type plasminogen activator with low efficiency. May play a specialized role in matrix remodeling processes in liver. Required to sense iron deficiency. Overexpression suppresses activation of the HAMP promoter.
Involvement in disease:
Defects in TMPRSS6 are the cause of iron-refractory iron deficiency anemia (IRIDA); also known as hypochromic microcytic anemia with defect in iron metabolism or hereditary iron-handling disorder or pseudo-iron-deficiency anemia. Key features include congenital hypochromic microcytic anemia, very low mean corpuscular erythrocyte volume, low transferrin saturation, abnormal iron absorption characterized by no hematologic improvement following treatment with oral iron, and abnormal iron utilization characterized by a sluggish, incomplete response to parenteral iron.
Function:
Serine protease which hydrolyzes a range of proteins including type I collagen, fibronectin and fibrinogen. Can also activate urokinase-type plasminogen activator with low efficiency. May play a specialized role in matrix remodeling processes in liver. Required to sense iron deficiency. Overexpression suppresses activation of the HAMP promoter.
Subcellular Location:
Cell membrane; Single-pass type II membrane protein.
Tissue Specificity:
Liver specific.
DISEASE:
Defects in TMPRSS6 are the cause of iron-refractory iron deficiency anemia (IRIDA) [MIM:206200]; also known as hypochromic microcytic anemia with defect in iron metabolism or hereditary iron-handling disorder or pseudo-iron-deficiency anemia. Key features include congenital hypochromic microcytic anemia, very low mean corpuscular erythrocyte volume, low transferrin saturation, abnormal iron absorption characterized by no hematologic improvement following treatment with oral iron, and abnormal iron utilization characterized by a sluggish, incomplete response to parenteral iron.
Similarity:
Belongs to the peptidase S1 family.
Contains 2 CUB domains.
Contains 3 LDL-receptor class A domains.
Contains 1 peptidase S1 domain.
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.