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Rabbit Anti-MASP2/RBITC Conjugated antibody (bs-1980R-RBITC)
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說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-1980R-RBITC
英文名稱 Rabbit Anti-MASP2/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標(biāo)記的甘露聚糖結(jié)合凝集素絲氨酸肽酶2抗體
別    名 mannan-binding lectin serine peptidase 2; MAP19; MASP-2; Mannose-binding protein-associated serine protease 2; MBL-associated serine protease 2; MASP1P1; sMAP; Small MBL associated protein.(Contains: Mannan-binding lectin serine protease 2 A chain; Mannan-binding lectin serine protease 2 B chain).  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 激酶和磷酸酶  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat,  (predicted: Horse, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 74kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MASP2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
MASP2, MBL associated serine protease 2, is a serum protease that plays an important role in the activation of the complement system via mannose-binding lectin (MBL). Binding of MBL to carbohydrate groups triggers MASP2 activation by auto-catalytic cleavage which leads to cleavage of C2 and C4, leading to their activation and to the formation of C3 convertase. MASP2 also binds to L-Ficolin and H-Ficolin, and when the complex binds pathogens the MASP is activated. Low MBL levels in serum are associated with increased susceptibility to infection, and an Asp105Gly mutation in MASP2 is associated with low MASP2 levels in serum. Reduced functional levels of MBL are also postulated to be associated with generation of autoantibodies in systemic lupus erythematosus, acting through a decreased clearance of apoptotic material. A truncated form of MASP2 that constitutes the amino 185 residues, Map19 is also a component of the MBL-MASP-1 complex.

Function:
Serum protease that plays an important role in the activation of the complement system via mannose-binding lectin. After activation by auto-catalytic cleavage it cleaves C2 and C4, leading to their activation and to the formation of C3 convertase.

Subunit:
Homodimer; disulfide-linked. Binds MBL2. Isoform 2 binds to MASP1. Binds SERPING1. Dimerization and MBL2 binding requires calcium ions.

Subcellular Location:
Secreted.

Tissue Specificity:
Plasma.

Post-translational modifications:
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
Activated by cleavage after Arg-444. The uncleaved zymogen is inactive towards synthetic substrates, but has sufficient activity to effect autocatalytic cleavage.

DISEASE:
Defects in MASP2 are the cause of MASP2 deficiency (MASPD) [MIM:613791]. MASPD is a disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease.

Similarity:
Belongs to the peptidase S1 family.
Contains 2 CUB domains.
Contains 1 EGF-like domain.
Contains 1 peptidase S1 domain.
Contains 2 Sushi (CCP/SCR) domains.

Database links:

Entrez Gene: 10747 Human

Omim: 605102 Human

SwissProt: O00187 Human

Unigene: 655645 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

甘露糖結(jié)合凝集素(MBL)是補(bǔ)體系統(tǒng)的原有成分,是由肝臟合成的C型凝集素并游離在血清中,也是鈣依賴性糖結(jié)合蛋白。
MBL可識(shí)別和結(jié)合病原微生物表面的甘露糖、巖藻糖和N-乙酰葡糖胺等糖結(jié)構(gòu)并首先與病原微生物的糖類配體結(jié)合,隨后構(gòu)象發(fā)生變化,激活與之相關(guān)聯(lián)的MBL相關(guān)的絲氨酸蛋白激酶(MASP),兩種MASP(MASP1、MASP2)具有與活化的C1s類似的生物學(xué)活性,其中MASP2可水解C4和C2分子,MASP1則可直接切割C3,而形成C3轉(zhuǎn)化酶,以至補(bǔ)體順序激活,這是補(bǔ)體激活的MBL途徑,這種補(bǔ)體順序的被激活在機(jī)體的固有性免疫防御中發(fā)揮重要作用。
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