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Matriptase 2 Rabbit pAb (bs-8296R)  
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50ul/1180.00元
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200ul/2800.00元
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產品編號 bs-8296R
英文名稱 Matriptase 2 Rabbit pAb
中文名稱 膜型絲氨酸蛋白酶2抗體
別    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.  
研究領域 細胞生物  免疫學  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat (predicted: Human,Mouse,Cow,Chicken,Dog)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 89 kDa
檢測分子量
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Matriptase 2: 711-811/811 <Extracellular>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
Q8IU80

Gene ID:
164656

產品圖片
Paraformaldehyde-fixed, paraffin embedded (rat liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incu
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