The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Predicted molecular weight: 76 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
Use a concentration of 5 µg/ml.
FunctionInhibitor of bone morphogenetic protein (BMP) function, it may regulate BMP responsiveness of osteoblasts and chondrocytes.
Tissue specificityHighly expressed in lung, and brain and also in primary chondrocytes.
Involvement in diseaseDefects 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.