title: Receptor-associated protein: a specialized chaperone and antagonist for members of the LDL receptor gene family.
abstract: Members of the LDL receptor gene family mediate cellular uptake of various extracellular ligands, including lipoprotein particles. Ligand interactions with these receptors can be antagonized by a 39 kDa receptor-associated protein. Recent biochemical, cellular, and genetic studies have shown that receptor-associated protein is a molecular chaperone/escort protein for LDL receptor-related protein, a member of the LDL receptor gene family that binds multiple ligands. These studies indicate that receptor-associated protein interacts with LDL receptor-related protein at multiple sites and assists the proper folding and disulfide bond formation of LDL receptor-related protein within the endoplasmic reticulum. Following the completion of folding, receptor-associated protein remains associated with the receptor during its subsequent trafficking along the early secretory pathway, thereby preventing premature ligand interaction with the receptor. The ability of receptor-associated protein to universally inhibit ligand interactions with members of the LDL receptor gene family underscores the use of this protein as a tool in the study of ligand-receptor interactions.
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, St. Louis, MO 63110, USA.
authors.forename: G
authors.initials: G
authors.lastname: Bu
mesh_terms: D006801:Humans; D008024:Ligands; D018832:Molecular Chaperones / Q000378:metabolism*; D011485:Protein Binding; D017510:Protein Folding; D011973:Receptors, LDL / Q000037:antagonists & inhibitors / Q000235:genetics / Q000378:metabolism*
chemical_list: D008024:Ligands; D018832:Molecular Chaperones; D011973:Receptors, LDL