FITC标记的膜型丝氨酸蛋白酶2抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的膜型丝氨酸蛋白酶2抗体

FITC标记的膜型丝氨酸蛋白酶2抗体

商家询价

产品名称： FITC标记的膜型丝氨酸蛋白酶2抗体

英文名称： Anti-Matriptase 2/FITC

产品编号： HZ-8296R-FITC

产品价格： null

产品产地： 中国/上海

品牌商标： HZbscience

更新时间： 2023-08-17T10:24:20

使用范围： ICC=1:50-200 IF=1:50-200

上海沪震实业有限公司
  • 联系人 : 鲍丽雯
  • 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
  • 邮编 : 200612
  • 所在区域 : 上海
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 Rabbit Anti-Matriptase 2/FITC Conjugated antibody 

FITC标记的膜型丝氨酸蛋白酶2抗体

 

产品编号 bs-8296R-FITC
英文名称 Anti-Matriptase 2/FITC
中文名称 FITC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
细胞定位 细胞膜 
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 application

丝氨酸蛋白酶水解一系列蛋白质，包括Ⅰ型胶原、纤维连接蛋白和纤维蛋白原。还可激活尿激酶型纤溶酶原激活剂，且效率低。可能在肝脏基质重塑过程中起特殊作用。需要检测缺铁。过表达抑制HAMP启动子的激活。

疾病参与：

 

TMPRSS6的缺陷是铁难治性缺铁性贫血（Irima）的原因；也被称为低色素性微囊性贫血，其中有铁代谢缺陷或遗传性铁处理障碍或假性缺铁性贫血。主要特征包括先天性低色素性微囊性贫血、极低的红细胞平均体积、低转铁蛋白饱和度、异常铁吸收，其特征是口服铁治疗后无血液改善，铁的异常利用特征为缓慢。对胃肠外铁的反应不完全。