The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
BMP binding endothelial regulator
BMP binding endothelial regulator precursor protein
BMP-binding endothelial regulator protein
Bone morphogenetic protein binding endothelial cell precursor derived regulator
Bone morphogenetic protein-binding endothelial cell precursor-derived regulator
Protein crossveinless 2
Inhibitor of bone morphogenetic protein (BMP) function, it may regulate BMP responsiveness of osteoblasts and chondrocytes.
Highly expressed in lung, and brain and also in primary chondrocytes.
Involvement in disease
Defects in BMPER are the cause of diaphanospondylodysostosis (DSD) [MIM:608022]. A rare, recessively inherited, perinatal lethal skeletal disorder. The primary skeletal characteristics of the phenotype include a small chest, abnormal vertebral segmentation, and posterior rib gaps containing incompletely differentiated mesenchymal tissue. Consistent craniofacial features include ocular hypertelorism, epicanthal folds, a depressed nasal bridge with a short nose, and low-set ears. The most commonly described extraskeletal finding is nephroblastomatosis with cystic kidneys, but other visceral findings have been described in some cases.