MMRN1,内皮细胞MMRN1蛋白抗体

产品编号HZ-9899R
英文名称MMRN1
中文名称内皮细胞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.
说 明 书0.1ml 0.2ml
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,
MMRN1,内皮细胞MMRN1蛋白抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量136kDa
细胞定位分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
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0.01M TBS(pH7.4) 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.
MMRN1,内皮细胞MMRN1蛋白抗体PubMedPubMed
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).

MMRN1,内皮细胞MMRN1蛋白抗体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. 

产品图片

Paraformaldehyde-fixed, paraffin embedded (rat bladder); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (MMRN1) Polyclonal Antibody, Unconjugated (HZ-9899R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining. Paraformaldehyde-fixed, paraffin embedded (rat bladder); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (MMRN1) Polyclonal Antibody, Unconjugated (HZ-9899R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining. Paraformaldehyde-fixed, paraffin embedded (mouse small intestine); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (MMRN1) Polyclonal Antibody, Unconjugated (HZ-9899R) at 1:400 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining. Paraformaldehyde-fixed, paraffin embedded (rat bladder); Antigen retrieval by boiling in sodium citrate buffer (pH6) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (MMRN1) Polyclonal Antibody, Unconjugated (HZ-9899R) at 1:400 overnight at 4°C, followed by a conjugated secondary (Goat Anti-rabbit IgG/Bio) for 20minutes at 37°C, followed by a conjugated streptavidin (HZ-0437P-Cy3) at[1:500] for 40 minutes and DAPI staining of the nuclei.