Prolow-density lipoprotein receptor-related protein 1

UniProt Q07954 Organism Homo sapiens Gene A2MR, APR, LRP1

Also known as: A2MR, APR, LRP1

Function

Endocytic receptor involved in endocytosis and in phagocytosis of apoptotic cells. Required for early embryonic development (By similarity). Involved in cellular lipid homeostasis. Involved in the plasma clearance of chylomicron remnants and activated LRPAP1 (alpha 2-macroglobulin), as well as the local metabolism of complexes between plasminogen activators and their endogenous inhibitors. Acts as an LRPAP1 alpha-2-macroglobulin receptor. Acts as TAU/MAPT receptor and controls the endocytosis of TAU/MAPT as well as its subsequent spread. May modulate cellular events, such as APP metabolism, kinase-dependent intracellular signaling, neuronal calcium signaling as well as neurotransmission. Also acts as a receptor for IGFBP3 to mediate cell growth inhibition.

Classification

Family (Pfam): PF12662 cEGF, PF07645 EGF_CA, PF14670 FXa_inhibition, PF00057 Ldl_recept_a, PF00058 Ldl_recept_b
InterPro: 6-blade_b-propeller_TolB-like, cEGF, EGF, EGF-like_Ca-bd_dom, EGF-type_Asp/Asn_hydroxyl_site, EGF_Ca-bd_CS, Growth_fac_rcpt_cys_sf, LDL_receptor-like_sf, LDLR-related, LDLR_class-A_CS, LDLR_classB_rpt, LDrepeatLR_classA_rpt, NOTCH1_EGF-like
Functional cluster: Immunoglobulin-Domain Cell Adhesion Proteins

Experimental structures · PDB · 7

1CR8 NMR
1D2L NMR
1J8E X-ray 1.85A
2FYJ NMR
2FYL NMR
2KNX NMR
2KNY NMR

A predicted model is available from AlphaFold.

Gene Ontology · 61

Disease associations

keratosis pilaris atrophicans MONDO:0018855
developmental dysplasia of the hip 3 MONDO:0958037

Drugs targeting this protein · 1

ANG1005 binding agent

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Prolow-density lipoprotein receptor-related protein 1 5 shared papers
Envelopment polyprotein 1 shared papers
Alpha-2-macroglobulin receptor-associated protein 3 shared papers
PTB-containing, cubilin and LRP1-interacting protein 1 shared papers
Disabled homolog 1 1 shared papers
Insulin-like growth factor-binding protein 3 1 shared papers
Exotoxin A 1 shared papers
Cubilin 1 shared papers
Sortilin-related receptor 1 shared papers
PTB domain-containing engulfment adapter protein 1 1 shared papers
Low-density lipoprotein receptor-related protein 4 1 shared papers
Cell death protein 6 1 shared papers

Literature · 39 cited papers

Multiple LDLR family members act as entry receptors for yellow fever virus. Nature · 2025
Heterozygous LRP1 deficiency causes developmental dysplasia of the hip by impairing triradiate chondrocytes differentiation due to inhibition of autophagy. Proc. Natl. Acad. Sci. U.S.A. · 2022
Lrp1 is a host entry factor for Rift Valley fever virus. Cell · 2021
LRP1 is a master regulator of tau uptake and spread. Nature · 2020
Whole exome sequencing identifies LRP1 as a pathogenic gene in autosomal recessive keratosis pilaris atrophicans. J. Med. Genet. · 2015
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
Diagnostic exome sequencing in persons with severe intellectual disability. N. Engl. J. Med. · 2012
Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry. J. Proteome Res. · 2009
A strategy for precise and large scale identification of core fucosylated glycoproteins. Mol. Cell. Proteomics · 2009
Identification of the ligands of protein interaction domains through a functional approach. Mol. Cell. Proteomics · 2007
The consensus coding sequences of human breast and colorectal cancers. Science · 2006
… and 27 more in the literature graph

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