Anti-EVC2 / Ellis-van Creveld syndrome protein 2

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. EVC2 antibody detects Ellis-van Creveld syndrome protein 2, a ciliary basal body protein required for Hedgehog signaling and skeletal development. The UniProt recommended name is Ellis-van Creveld syndrome protein 2 (EVC2). Also known as Limbin, this protein anchors signaling complexes at the base of the primary cilium, coordinating receptor localization and developmental signal transduction.Functionally, EVC2 antibody identifies a 1,285-amino-acid transmembrane protein localized to the ciliary pocket region. EVC2 forms a complex with EVC, tethering Hedgehog pathway components such as Smoothened and Gli transcription factors at the cilium base to modulate downstream transcriptional activity. It is essential for skeletal morphogenesis and growth plate function.The EVC2 gene is located on chromosome 4p16.2 and is expressed in ciliated tissues, including cartilage, bone, and embryonic organs. Proper EVC2 function maintains ciliary integrity and ensures controlled Hedgehog pathway activation during tissue differentiation.Pathologically, mutations in EVC2 cause Ellis-van Creveld syndrome and Weyers acrofacial dysostosis, characterized by short stature, polydactyly, and craniofacial abnormalities. Disruption of EVC2 impairs Hedgehog signaling, leading to developmental malformations. Research using EVC2 antibody supports studies in ciliopathies, developmental biology, and skeletal genetics.EVC2 antibody is validated for western blotting, immunofluorescence, and immunohistochemistry to detect ciliary and developmental proteins. NSJ Bioreagents provides EVC2 antibody reagents optimized for cilia structure, signaling, and morphogenesis research.Structurally, Ellis-van Creveld syndrome protein 2 is a membrane-tethered scaffold with coiled-coil domains that mediate interaction with EVC and ciliary membrane components. This antibody enables examination of EVC2's function in Hedgehog signaling and developmental regulation.