产品名称
MMRN1 Rabbit Polyclonal Antibody
基因名称
MMRN1 ECM EMILIN4 GPIA* MMRN
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/22915
Human Swissprot No.
Q13201
Human Swissprot Link
https://www.uniprot.org/uniprotkb/Q13201/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/70945
Mouse Swissprot No.
B2RPV6
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/B2RPV6
免疫原
Synthesized peptide derived from human MMRN1 AA range: 963-1013
特异性
This antibody detects endogenous levels of MMRN1 at Human/Mouse
宿主
Polyclonal, Rabbit,IgG
背景介绍
Multimerin is a massive, soluble protein found in platelets and in the endothelium of blood vessels. It is comprised of subunits linked by interchain disulfide bonds to form large, variably sized homomultimers. Multimerin is a factor V/Va-binding protein and may function as a carrier protein for platelet factor V. It may also have functions as an extracellular matrix or adhesive protein. Recently, patients with an unusual autosomal-dominant bleeding disorder (factor V Quebec) were found to have a deficiency of platelet multimerin. [provided by RefSeq, Jul 2008],
组织表达
Synthesized by endothelial cells and megakaryocytes. Stored in platelet alpha granules and endothelial cell Weibel-Palade bodies, following activation of these cells, it is released and attached to megakaryocytes, platelets, endothelium and subendothelium of blood vessels. Not found in plasma. Found in vascular tissues such as placenta, lung, and liver.
功能
disease:Deficiency in multimerin-1 due to proteolytic degradation within the platelet alpha granules is associated with an autosomal dominant bleeding disorder (factor V Quebec).,function:Carrier protein for platelet (but not plasma) factor V/Va. Plays a role in the storage and stabilization of factor V in platelets. Upon release following platelet activation, may limit platelet and plasma factor Va-dependent thrombin generation. Ligand for integrin alpha-IIb/beta-3 and integrin alpha-V/beta-3 on activated platelets, and may function as an extracellular matrix or adhesive protein.,PTM:Extensively N-glycosylated.,PTM:The N-terminus is blocked.,similarity:Contains 1 C1q domain.,similarity:Contains 1 EGF-like domain.,similarity:Contains 1 EMI domain.,subunit:Multimeric. Composed of varying sized, disulfide-linked multimers, the smallest of which is a homotrimer. Proteolysis of the promultimerin in the N-terminal region, leads to the mature p155 form that is stored in platelets. Interacts with factor V/Va.,tissue specificity:Synthesized by endothelial cells and megakaryocytes. Stored in platelet alpha granules and endothelial cell Weibel-Palade bodies, following activation of these cells, it is released and attached to megakaryocytes, platelets, endothelium and subendothelium of blood vessels. Not found in plasma. Found in vascular tissues such as placenta, lung, and liver.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
