low density lipoprotein (LDL) receptor related protein (LRP) 2 (LRP2, glycoprotein 330, gp330)

Function: - acts together with cubilin to mediate HDL endocytosis (putative) - may participate in regulation of parathyroid hormone & parathyroid-hormone-related protein release - binds plasminogen, extracellular matrix components, plasminogen activator-plasminogen activator inhibitor type 1 complex, apolipoprotein E-enriched beta-VLDL, lipoprotein lipase, lactoferrin, CLU/clusterin & Ca⁺² - forms a multimeric complex together with a receptor-associated protein (RAP) - binds to ankyrin-repeat family A protein 2 (ANKRA2) - interacts with LRP2BP Structure: - belongs to the LDLR family - contains 17 EGF-like domains - contains 36 LDL-receptor class A domains - contains 37 LDL-receptor class B repeats Compartment: membrane Expression: absorptive epithelia, including renal proximal tubules Pathology: - defects in LRP2 are the cause of Donnai-Barrow syndrome

General

glycoprotein low-density lipoprotein (LDL) receptor related protein (LRP)

Properties

SIZE: entity length = 4655 aa MW = 522 kD COMPARTMENT: cellular membrane MOTIF: signal sequence {1-25} LDL-receptor class A {26-3964} (36) MOTIF: cysteine residue {C28} MODIFICATION: cysteine residue {C40} cysteine residue {C35} MODIFICATION: cysteine residue {C53} cysteine residue {C40} MODIFICATION: cysteine residue {C28} cysteine residue {C47} MODIFICATION: cysteine residue {C62} cysteine residue {C53} MODIFICATION: cysteine residue {C35} cysteine residue {C62} MODIFICATION: cysteine residue {C47} EGF domain {309-4411} (17) MOTIF: cysteine residue {C312} MODIFICATION: cysteine residue {C321} cysteine residue {C317} MODIFICATION: cysteine residue {C330} cysteine residue {C321} MODIFICATION: cysteine residue {C312} cysteine residue {C330} MODIFICATION: cysteine residue {C317} cysteine residue {C332} MODIFICATION: cysteine residue {C346} N-glycosylation site {N341} cysteine residue {C346} MODIFICATION: cysteine residue {C332} N-glycosylation site {N388} LDL-receptor {436-4283} (37) MOTIF: N-glycosylation site {N463} N-glycosylation site {N1064} N-glycosylation site {N1464} N-glycosylation site {N3211} N-glycosylation site {N4327} transmembrane domain {4424-4446} SH3-binding site NAME: SH3-binding site SITE: 4453-4459 SH3-binding site NAME: SH3-binding site SITE: 4456-4462 Ser phosphorylation site {S4463} Ser phosphorylation site {S4471} Endocytosis signal {4521-4526} Endocytosis signal {4593-4598} SH2-binding {4598-4601} SH3-binding site NAME: SH3-binding site SITE: 4611-4617 SH3-binding site NAME: SH3-binding site SITE: 4616-4622

Database Correlations

OMIM correlations MORBIDMAP 600073 UniProt P98164 PFAM correlations Entrez Gene 4036 Kegg hsa:4036