Extracellular matrix protein 1 is an extracellular protein containing motifs with a cysteine pattern characteristic of the cysteine pattern of the ligand-binding "double-loop" domains of the albumin protein family. This gene maps outside of the epidermal differentiation complex (EDC), a cluster of three gene families involved in epidermal differentiation. Alternatively spliced transcript variants encoding distinct isoforms have been described.Defects in ECM1 are the cause of lipoid proteinosis 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.

Mouse Extracellular matrix protein 1 (ECM1) ELISA Kit employs a two-site sandwich ELISA to quantitate ECM1 in samples. An antibody specific for ECM1 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyECM1 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for ECM1 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of ECM1 bound in the initial step. The color development is stopped and the intensity of the color is measured.

Mouse Extracellular matrix protein 1 (ECM1) ELISA Kit listed herein is for research use only and is not intended for use in human or clinical diagnosis. Suggested applications of our products are not recommendations to use our products in violation of any patent or as a license. We cannot be responsible for patent infringements or other violations that may occur with the use of this product.

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