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anti-ECM1 antibody :: Rabbit ECM1 Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS127659
Western Blot (WB)
Unit / Price
0.05 mL  /  $205 +1 FREE 8GB USB
0.1 mL  /  $320 +1 FREE 8GB USB
0.2 mL  /  $605 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

ECM1, Polyclonal Antibody

★Popular Item★
 Also Known As   

ECM1 Polyclonal Antibody

 Product Synonym Names    ECM1; extracellular matrix protein 1; Secretory component p85
 Product Gene Name   

anti-ECM1 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    phenotype 602201
 3D Structure    ModBase 3D Structure for Q16610
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Purity/Purification    Affinity Purification
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 Species    Human
 Immunogen    Recombinant protein of human ECM1
 Buffer    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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 Preparation and Storage    Store at -20 degree C. Avoid freeze / thaw cycles.
 Other Notes    Small volumes of anti-ECM1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for anti-ECM1 antibody

   ECM1 (extracellular matrix protein 1), also known as secretory component p85, is a secreted glycoprotein that is essential for the proper structure and function of the skin. It is widely expressed and localizes to the extracellular matrix. ECM1 binds to a variety of extracellular matrix components, including Perlecan, Fibulin and matrix metalloproteinase-9 (MMP-9), and participates in the structural organization of the dermis. In addition, ECM1 enhances the association of Collagen Type IV with Laminin 332 suggesting that it is a key player in interstitial dermis and the dermal-epidermal junction. Mutations in the gene encoding ECM1 result in the autosomal recessive disorder lipoid proteinosis (LiP). LiP is characterized by hyalinization of the dermis and reduplication of the basement membrane of the skin. LiP patients exhibit thickening of the skin and mucosae. Four splice variants (known as ECM1a-ECM1d) exist for ECM1.
 Product Categories/Family for anti-ECM1 antibody    Polyclonal
 Applications Tested/Suitable for anti-ECM1 antibody   

Western Blot (WB), Immunohistochemistry (IHC)

 Application Notes for anti-ECM1 antibody    WB: 1:500 - 1:1000
IHC: 1:50 - 1:100
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 Western Blot (WB) of anti-ECM1 antibody    Western blot analysis of extracts of various cell lines, using ECM1 antibody. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane.
Blocking Buffer: 3% nonfate dry milk in TBST.
anti-ECM1 antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for ECM1. It may not necessarily be applicable to this product.
 NCBI GI #    48429255
 NCBI GeneID    1893
 NCBI Accession #    Q16610.2 [Other Products]
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 UniProt Primary Accession #    Q16610 [Other Products]
 UniProt Secondary Accession #    O43266; Q5T5G4; Q5T5G5; Q5T5G6; Q8IZ60; A8K8S0; B4DW49; B4DY60 [Other Products]
 UniProt Related Accession #    Q16610 [Other Products]
 Molecular Weight    Observed MW: 55kDa
Calculated MW: 19kDa, 46kDa, 60kDa, 63kDa
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 NCBI Official Full Name    Extracellular matrix protein 1
 NCBI Official Synonym Full Names    extracellular matrix protein 1
 NCBI Official Symbol    ECM1 [Similar Products]
 NCBI Official Synonym Symbols   
URBWD
[Similar Products]
 NCBI Protein Information    extracellular matrix protein 1; secretory component p85
 UniProt Protein Name    Extracellular matrix protein 1
 UniProt Synonym Protein Names   
Secretory component p85
 Protein Family    Extracellular matrix protein
 UniProt Gene Name    ECM1 [Similar Products]
 UniProt Entry Name    ECM1_HUMAN
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 NCBI Summary for ECM1    This gene encodes a soluble protein that is involved in endochondral bone formation, angiogenesis, and tumor biology. It also interacts with a variety of extracellular and structural proteins, contributing to the maintenance of skin integrity and homeostasis. Mutations in this gene are associated with lipoid proteinosis disorder (also known as hyalinosis cutis et mucosae or Urbach-Wiethe disease) that is characterized by generalized thickening of skin, mucosae and certain viscera. Alternatively spliced transcript variants encoding distinct isoforms have been described for this gene. [provided by RefSeq, Feb 2011]
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 UniProt Comments for ECM1    ECM1: Involved in endochondral bone formation as negative regulator of bone mineralization. Stimulates the proliferation of endothelial cells and promotes angiogenesis. Inhibits MMP9 proteolytic activity. Defects in ECM1 are the cause of lipoid proteinosis (LiP); also known as lipoid proteinosis of Urbach and Wiethe or hyalinosis cutis et mucosae. LiP is a rare autosomal recessive disorder characterized by generalized thickening of skin, mucosae and certain viscera. Classical features include beaded eyelid papules and laryngeal infiltration leading to hoarseness. Histologically, there is widespread deposition of hyaline material and disruption/reduplication of basement membrane. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 1q21

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space

Molecular Function: protein C-terminus binding; signal transducer activity; protein binding; enzyme binding; protease binding; interleukin-2 receptor binding; laminin binding

Biological Process: ossification; positive regulation of I-kappaB kinase/NF-kappaB cascade; negative regulation of peptidase activity; signal transduction; regulation of transcription from RNA polymerase II promoter; negative regulation of bone mineralization; positive regulation of angiogenesis; biomineral formation; negative regulation of cytokine and chemokine mediated signaling pathway; positive regulation of endothelial cell proliferation; angiogenesis; regulation of T-helper 2 type immune response; inflammatory response

Disease: Lipoid Proteinosis Of Urbach And Wiethe
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 Research Articles on ECM1    1. homozygous missense mutation p.C220G of ECM1 was identified by Sanger sequencing, which is a major allele in Chinese patients with LP
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Diseases associated with anti-ECM1 antibodyOrgans/Tissues associated with anti-ECM1 antibody
 Disease Name  Pubmed Publications
 Lipoid Proteinosis of Urbach and Wiethe Antibodies  >50 publications with ECM1 and Lipoid Proteinosis of Urbach and Wiethe
 Carcinoma Antibodies  >21 publications with ECM1 and Carcinoma
 Breast Neoplasms Antibodies  >10 publications with ECM1 and Breast Neoplasms
 Inflammation Antibodies  >9 publications with ECM1 and Inflammation
 Colitis, Ulcerative Antibodies  >9 publications with ECM1 and Colitis, Ulcerative
 Carcinoma, Squamous Cell Antibodies  >6 publications with ECM1 and Carcinoma, Squamous Cell
 Adenocarcinoma Antibodies  >5 publications with ECM1 and Adenocarcinoma
 Disease Models, Animal Antibodies  >3 publications with ECM1 and Disease Models, Animal
 Precancerous Conditions Antibodies  >2 publications with ECM1 and Precancerous Conditions
 Neurobehavioral Manifestations Antibodies  >2 publications with ECM1 and Neurobehavioral Manifestations
 Organ/Tissue Name  Pubmed Publications
 Skin Antibodies  >57 publications with ECM1 and Skin
 Blood Antibodies  >26 publications with ECM1 and Blood
 Vascular Antibodies  >18 publications with ECM1 and Vascular
 Bone Antibodies  >10 publications with ECM1 and Bone
 Brain Antibodies  >9 publications with ECM1 and Brain
 Lymph Node Antibodies  >7 publications with ECM1 and Lymph Node
 Muscle Antibodies  >6 publications with ECM1 and Muscle
 Thyroid Antibodies  >5 publications with ECM1 and Thyroid
 Connective Tissue Antibodies  >5 publications with ECM1 and Connective Tissue
 Mouth Antibodies  >5 publications with ECM1 and Mouth
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