The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
% SDS-PAGE. ab123754 is purified by proprietary chromatographic techniques. Purity is greater than 95% as determined by HPLC and SDS-PAGE.
Centrifuge the vial prior to opening.
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Preparation and Storage
Stability and Storage
Shipped at 4°C. Store at -20ºC.
Centrifuge the vial prior to opening. Reconstitute in sterile PBS to a concentration of 100 µg/ml. This solution can then be diluted into other aqueous buffers.
Reconstituted ab123754 should be stored at 4°C for 2-7 days and at -20°C for future use. For long term storage it is recommended to add a carrier protein (0.1 % HSA or BSA). Avoid freeze/thaw cycles.
Osler Rendu Weber syndrome 1
Major glycoprotein of vascular endothelium. May play a critical role in the binding of endothelial cells to integrins and/or other RGD receptors.
Endoglin is restricted to endothelial cells in all tissues except bone marrow.
Involvement in disease
Defects in ENG are the cause of hereditary hemorrhagic telangiectasia type 1 (HHT1) [MIM:187300, 108010]; also known as Osler-Rendu-Weber syndrome 1 (ORW1). HHT1 is an autosomal dominant multisystemic vascular dysplasia, characterized by recurrent epistaxis, muco-cutaneous telangiectases, gastro-intestinal hemorrhage, and pulmonary (PAVM), cerebral (CAVM) and hepatic arteriovenous malformations; all secondary manifestations of the underlying vascular dysplasia. Although the first symptom of HHT1 in children is generally nose bleed, there is an important clinical heterogeneity.