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Product Name Anti-MASP2/Cy7 Chinese Name Cy7标记的甘露聚糖结合凝集素丝氨酸肽酶2抗体 Alias 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). Research Area Kinases and Phosphatases Immunogen Species Rabbit Clonality Polyclonal React Species Human, Mouse, Rat, (predicted: Horse, ) Applications
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 74kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human MASP2 Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage 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. Product Detail 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.
甘露糖结合凝集素(MBL)是补体系统的原有成分,是由肝脏合成的C型凝集素并游离在血清中,也是钙依赖性糖Binding protein。
MBL可识别和结合病原微生物表面的甘露糖、岩藻糖和N-乙酰葡糖胺等糖结构并首先与病原微生物的糖类配体结合,随后构象发生变化,激活与之相关联的MBL相关的丝氨酸蛋白激酶(MASP),两种MASP(MASP1、MASP2)具有与活化的C1s类似的生物学活性,其中MASP2可水解C4和C2分子,MASP1则可直接切割C3,而形成C3转化酶,以至补体顺序激活,这是补体激活的MBL途径,这种补体顺序的被激活在机体的固有性免疫防御中发挥重要作用。
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