ECM1, Monoclonal Antibody

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

Monoclonal

IgG1

Mouse

This antibody can be stored at 2 degree C- 8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -70 degree C. Preservative-Free. Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2015 Certified Laboratory.

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.

ELISA (EIA)

ELISA: 0.5-1 mug/mL. This antibody can be used at 0.5-1 mug/mL with the appropriate secondary reagents to detect Human ECM1. The detection limit for Human ECM1 is approximately 0.16 ng/well.

63,563 Da

extracellular matrix protein 1

extracellular matrix protein 1

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; extracellular region

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

Biological Process: negative regulation of bone mineralization; negative regulation of peptidase activity; platelet degranulation; positive regulation of angiogenesis; positive regulation of endothelial cell proliferation; positive regulation of I-kappaB kinase/NF-kappaB cascade

Disease: Lipoid Proteinosis Of Urbach And Wiethe

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