Multimerin 1 (MMRN1)

ECM; EMILIN4; GPIa; MMRN; Glycoprotein Ia; Elastin microfibril interface located protein 4; Endothelial cell multimerin; Platelet glycoprotein Ia; 155 kDa platelet multimerin

SPN	U229
HGNC	7178
MGI	1918195
PubMed	19132231
RGD	1587248
UniProt	Q13201
Wiki	MMRN1

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 were found to have a deficiency of platelet multimerin.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.

Reference

"The cDNA sequence of human endothelial cell multimerin. A unique protein with RGDS, coiled-coil, and epidermal growth factor-like domains and a carboxyl terminus similar to the globular domain of complement C1q and collagens type VIII and X."
J. Biol. Chem. 270:18246-18251(1995) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"Generation and annotation of the DNA sequences of human chromosomes 2 and 4."
Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Platelet glycoprotein Ia* is the processed form of multimerin --isolation and determination of N-terminal sequences of stored and released forms."
Thromb. Haemost. 80:645-648(1998) [PubMed] [Europe PMC] [Abstract]

"Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides."
Nat. Biotechnol. 21:566-569(2003) [PubMed] [Europe PMC] [Abstract]

"Multimerin is found in the alpha-granules of resting platelets and is synthesized by a megakaryocytic cell line."
J. Clin. Invest. 91:2630-2639(1993) [PubMed] [Europe PMC] [Abstract]

"Platelet multimerin and its proteolytic processing."
Thromb. Haemost. 82:1779-1780(1999) [PubMed] [Europe PMC] [Abstract]

"Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]

"Analyses of cellular multimerin 1 receptors: in vitro evidence of binding mediated by alphaIIbbeta3 and alphavbeta3."
Thromb. Haemost. 94:1004-1011(2005) [PubMed] [Europe PMC] [Abstract]

"Elucidation of N-glycosylation sites on human platelet proteins: a glycoproteomic approach."
Mol. Cell. Proteomics 5:226-233(2006) [PubMed] [Europe PMC] [Abstract]

"Multimerin 1 binds factor V and activated factor V with high affinity and inhibits thrombin generation."
Thromb. Haemost. 100:1058-1067(2008) [PubMed] [Europe PMC] [Abstract]

"An autosomal dominant, qualitative platelet disorder associated with multimerin deficiency, abnormalities in platelet factor V, thrombospondin, von Willebrand factor, and fibrinogen and an epinephrine aggregation defect."
Blood 87:4967-4978(1996) [PubMed] [Europe PMC] [Abstract]

"Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]

"A strategy for precise and large scale identification of core fucosylated glycoproteins."
Mol. Cell. Proteomics 8:913-923(2009) [PubMed] [Europe PMC] [Abstract]