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货号： bs-9899R-Gold 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-9899R-Gold
- 英文名称
- Anti-MMRN1/Gold
- 中文名称
- 胶体金标记的内皮细胞MMRN1蛋白抗体
- 别 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul（10nm 15nm 35nm）
- 研究领域
- 心血管 细胞外基质
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Cow, Horse, Sheep,
- 产品应用
- IEM=1:20-200 IGS=1:20-200 GICA=1:20-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 136kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human MMRN1
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- 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.
- 产品介绍
- 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.