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.
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.
Belongs to the leprecan family. Contains 1 Fe2OG dioxygenase domain. Contains 4 TPR repeats.
O-glycosylated; chondroitin sulfate.
Endoplasmic reticulum. Secreted > extracellular space > extracellular matrix. Secreted into the extracellular matrix as a chondroitin sulfate proteoglycan.
SDS-PAGE - LEPRE1 protein (Tagged-His Tag) (ab92037)
The image shows an electrophoretic assay performed using an Agilent 5100 ALP. In some images coloured control bands can be seen at 15 kDa (green) and/or 240 kDa (purple). The protein-specific band is blue.
Recombinant Human LEPRE1 protein (ab92037)参考文献
has not yet been referenced specifically in any publications.