ECM1 Polyclonal Antibody | MyBioSource MBS127659 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

ECM1 Polyclonal Antibody

catalog :

MBS127659

quantity :

0.05 mL

price :

235 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, immunohistochemistry

more info or order :

MyBioSource product webpage

image

image 1 :

Western blot analysis of extracts of various cell lines, using ECM1 antibody. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST.

product information

catalog number :

MBS127659

products type :

Antibody

products full name :

ECM1 Polyclonal Antibody

products short name :

[ECM1]

products name syn :

[ECM1; extracellular matrix protein 1; Secretory component p85]

other names :

[Extracellular matrix protein 1; Extracellular matrix protein 1; extracellular matrix protein 1; secretory component p85; extracellular matrix protein 1; Secretory component p85]

products gene name :

[ECM1]

other gene names :

[ECM1; ECM1; URBWD]

uniprot entry name :

ECM1_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human, Mouse, Rat

sequence length :

540

purity :

Affinity Purification

storage stability :

Store at -20 degree C. Avoid freeze / thaw cycles.

tested application :

Western Blot (WB), Immunohistochemistry (IHC)

app notes :

WB: 1:500 - 1:1000. IHC: 1:50 - 1:100

image1 heading :

Western Blot (WB)

other info1 :

Species: Human

other info2 :

Immunogen: Recombinant protein of human ECM1. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

products categories :

Polyclonal

products description :

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.

ncbi gi num :

48429255

ncbi acc num :

Q16610.2

uniprot acc num :

Q16610

ncbi mol weight :

Observed MW: 55kDa. Calculated MW: 19kDa, 46kDa, 60kDa, 63kDa

ncbi summary :

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]

uniprot summary :

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

size1 :

0.05 mL

price1 :

235 USD

size2 :

0.1 mL

price2 :

350

more info or order :

MyBioSource product webpage