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Rabbit Anti-MMRN1/AP Conjugated antibody (bs-9899R-AP)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-9899R-AP
英文名稱 Rabbit Anti-MMRN1/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Sheep, )
產品應用 WB=1:50-200 IHC-P=1:50-200 IHC-F=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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