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    Rabbit Anti-MMRN1/RBITC Conjugated antibody (bs-9899R-RBITC)
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    說(shuō) 明 書(shū): 100ul  
    100ul/2980.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-9899R-RBITC
    英文名稱(chēng) Rabbit Anti-MMRN1/RBITC Conjugated antibody
    中文名稱(chēng) 羅丹明(RBITC)標記的內皮細胞MMRN1蛋白抗體
    別    名 ECM; Elastin microfibril interface located protein 4; Elastin microfibril interfacer 4; EMILIN 4; EMILIN4; Endothelial cell multimerin 1; Glycoprotein Ia; GlycoproteinIa; GPIa; MMRN 1; MMRN; Multimerin 1; Multimerin-1; Multimerin1; MMRN1_HUMAN.  
    規格價(jià)格 100ul/2980元 購買(mǎi)        大包裝/詢(xún)價(jià)
    說(shuō) 明 書(shū) 100ul  
    研究領(lǐng)域 心血管  細胞外基質(zhì)  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    交叉反應 (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Sheep, )
    產(chǎn)品應用 IF=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 136kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human MMRN1
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 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:
    Multimerin-1 is a 1,228 amino acid secreted protein that contains one C1q domain, one EMI domain and one EGF-like domain. Synthesized in megakaryocytes and endothelial cells and present in liver, lung and placenta, Multimerin-1 exists as a multimeric structure composed of varying disulfide-linked multimers and functions as a carrier protein for platelet factors (specifically platelet factor V), playing a role in the stabilization and storage of factor V in platelets. In addition, Multimerin-1 acts as a ligand for select Integrins and may participate in extracellular matrix adhesion. Defects in the gene encoding Multimerin-1 that lead to Multimerin-1 deficiency are associated with autosomal dominant bleeding disorders due to platelet factor malfunction. Multiple isoforms of Multimerin-1 exist due to alternative splicing events.

    Function:
    Carrier protein for platelet (but not plasma) factorV/Va. Plays a role in the storage and stabilization of factor V inplatelets. Upon release following platelet activation, may limitplatelet and plasma factor Va-dependent thrombin generation. Ligandfor integrin alpha-IIb/beta-3 and integrin alpha-V/beta-3 onactivated platelets, and may function as an extracellular matrix oradhesive protein.

    Subunit:
    Multimeric. Composed of varying sized, disulfide-linkedmultimers, the smallest of which is a homotrimer. Proteolysis ofthe promultimerin in the N-terminal region, leads to the maturep155 form that is stored in platelets. Interacts with factor V/Va.

    Subcellular Location:
    Secreted (Potential).

    Tissue Specificity:
    MMRN1 (Multimerin 1) is a factor V/Va-binding protein and may function as a carrier protein for platelet (but not plasma) factor V/Va. It is found in platelets, megakaryocytes, endothelium and extracellular matrix fibers, but not in plasma.

    Post-translational modifications:
    The N-terminus is blocked.
    Extensively N-glycosylated.

    DISEASE:
    Note=Deficiency in multimerin-1 due to proteolyticdegradation within the platelet alpha granules is associated withan autosomal dominant bleeding disorder (factor V Quebec).

    Similarity:
    Contains 1 C1q domain.
    Contains 1 EGF-like domain.
    Contains 1 EMI domain.

    Database links:

    Entrez Gene: 22915 Human

    Omim: 601456 Human

    SwissProt: Q13201 Human

    Unigene: 268107 Human



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