Mouse Limbin (EVC2) ELISA kit; LBN; limbin; Ellis van Creveld syndrome 2

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

Store all reagents at 2-8 degree C

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Small volumes of EVC2 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.

MBS9327526 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Ellis van Creveld syndrome 2 (EVC2) 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 EVC2. The ELISA analytical biochemical technique of the MBS9327526 kit is based on EVC2 antibody-EVC2 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect EVC2 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, EVC2. 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(%).

137,639 Da

Ellis van Creveld syndrome 2

limbin; Ellis van Creveld syndrome 2 homolog

Limbin

Lbn??[Similar Products]

LBN_MOUSE

EVC2: Positive regulator of the hedgehog signaling pathway. Plays a critical role in bone formation and skeletal development. Defects in EVC2 are a cause of Ellis-van Creveld syndrome (EVC); also known as chondroectodermal dysplasia. EVC is an autosomal recessive disorder characterized by the clinical tetrad of chondrodystrophy, polydactyly, ectodermal dysplasia and cardiac anomalies. Patients manifest short-limb dwarfism, short ribs, postaxial polydactyly and dysplastic nails and teeth. Congenital heart defects, most commonly an atrioventricular septal defect, are observed in 60% of affected individuals. Defects in EVC2 are a cause of acrofacial dysostosis Weyers type (WAD); also known as Curry-Hall syndrome. Acrofacial dysostoses are a heterogeneous group of disorders combining limb defects with facial abnormalities. WAD is an autosomal dominant disorder characterized by dysplastic nails, postaxial polydactyly, acrofacial dysostosis, short limbs and short stature. The phenotype is milder than Ellis-van Creveld syndrome. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral

Cellular Component: cytoskeleton; cell projection; membrane; cytoplasm; integral to membrane; plasma membrane; nucleus; cilium

Molecular Function: protein binding

Biological Process: smoothened signaling pathway

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