Extracellular Matrix Protein 1, ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 1; NC_000001.10 (150480487..150486265). Location: 1q21

Store all reagents at 2-8 degree C

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Small volumes of ECM1 elisa kit 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.

MBS019255 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Extracellular Matrix Protein 1 (ECM1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing ECM1. The ELISA analytical biochemical technique of the MBS019255 kit is based on ECM1 antibody-ECM1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect ECM1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, ECM1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

60,674 Da

extracellular matrix protein 1

extracellular matrix protein 1; secretory component p85

Extracellular matrix protein 1

ECM1_HUMAN

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]

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; proteinaceous extracellular matrix; extracellular space

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

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

Disease: Lipoid Proteinosis Of Urbach And Wiethe

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