LEPRE1 Antibody – Mouse Polyclonal

LEPRE1: 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). 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. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Oxidoreductase; EC 1.14.11.7; Secreted; Cell development/differentiation; Secreted, signal peptide

Cellular Component: proteinaceous extracellular matrix; membrane; endoplasmic reticulum; cytoplasm; plasma membrane; extracellular region; basement membrane; nucleus

Molecular Function: procollagen-proline 3-dioxygenase activity; oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen; dioxygenase activity; L-ascorbic acid binding; iron ion binding; metal ion binding; oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors; oxidoreductase activity

Biological Process: regulation of protein secretion; collagen fibril organization; collagen metabolic process; protein stabilization; protein folding; peptidyl-proline hydroxylation; protein amino acid hydroxylation; cell growth; regulation of ossification