catalog number :
MBS074772
products type :
ELISA Kit
products full name :
Camel Extracellular Matrix Protein 1 ELISA Kit
products short name :
[Extracellular Matrix Protein 1]
other names :
[extracellular matrix protein 1 isoform 3; 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
specificity :
No significant cross-reactivity or interference between Camel ECM1 and analogues was observed.
storage stability :
Store all reagents at 2-8 degree C
other info1 :
Samples: Serum, Plasma, Tissue Homogenate, Feces and Urine. Assay Type: Sandwich. Detection Range: 62.5 pg/ml - 2000 pg/ml. Sensitivity: 10 pg/ml.
other info2 :
Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].
products description :
Intended Uses: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! It is intended to be determinated ECM1 concentrations in Camel serum, plasma and other body fluids. Using Purified Camel ECM1 antibody to coat Microelisa Stripplate wells to make solid-phase antibody, then add ECM1 and ECM1 antibody which has been labeled with HRP to wells, then the reactants become antibody-antigen-antibody-enzyme complex, after washing completely, add TMB substrate solution, TMB substrate becomes blue color under HRP enzyme-catalyzed, reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. The concentration of ECM1 in the samples is then determined by comparing the O.D. of the samples to the standard curve.
ncbi acc num :
NP_001189787.1
ncbi gb acc num :
NM_001202858.1
ncbi mol weight :
60,674 Da
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; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 1q21. Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix. Molecular Function: protein C-terminus binding; protein binding; signal transducer activity; 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
size4 :
10x96-Strip-Wells