The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
追加情報WB: Use at a concentration of 1-5 µg/ml. IHC-P: Use at a concentration of 6 µg/ml.
Not yet tested in other applications. Optimal dilutions/concentrations should be determined by the end user.
機能Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts.
関連疾患Defects in LEPRE1 are the cause of osteogenesis imperfecta type 8 (OI8) [MIM:610915]. A connective tissue disorder characterized by disproportionate short stature, severe osteoporosis, shortening of the long bones, white sclerae, a round face and a short barrel-shaped chest.
配列類似性Belongs to the leprecan family. Contains 1 Fe2OG dioxygenase domain. Contains 4 TPR repeats.
翻訳後修飾O-glycosylated; chondroitin sulfate.
細胞内局在Endoplasmic reticulum. Secreted > extracellular space > extracellular matrix. Secreted into the extracellular matrix as a chondroitin sulfate proteoglycan.