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.
Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:50-400Immunocytochemistry: 1:50-500Immunohistochemistry (frozen): 1:50-500Immunohistochemistry (paraffin): 1:10-100ELISA: 1:100-1:5000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：200ul
Host：rabbit
Source Antibody：mouse
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes mouse ECM1.
Isotype：IgG
Calc Applications Abbrev：E IC IHC WB
Calc Crossreactivity：Mo
Immunogen：Recombinant protein corresponding to Ala20-Glu434 of mouse ECM1, fused to His-Tag and GST-tag at N-terminal expressed in E. coli.
