Rabbit Anti-MMRN1/FITC Conjugated antibody

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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.

Cat:

SL9899R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,Cow,Horse,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human MMRN1

Format：

Lyophilized or Liquid

Storage instructions：

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 ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

136kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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: Q1641 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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