LDLR_HUMAN » Low-density lipoprotein receptor

LDLR_HUMAN » Low-density lipoprotein receptor
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
LDLR_HUMAN » Low-density lipoprotein receptor » LDL receptor;
Hydrophobic Thickness 34.8 ± 3.8 Å
Tilt Angle 1 ± 0°
ΔGtransfer -30.0 kcal/mol
ΔGfold -21.6 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, Reactome, HMDB
Topology Out
TM Segments 789-812 (784-816)
Pathways

Bile secretion (KEGG)

Endocytosis (KEGG)

Hepatitis C (KEGG)

Ovarian steroidogenesis (KEGG)

Signal Transduction (Reactome)

Toxoplasmosis (KEGG)

PDB 2lgp (144-235), 1ajj (196-232), 1ldl (20-67), 1f5y (22-104), 1n7d (22-720), 1d2j (233-272), 1f8z (234-272), 1xfe (272-353), 2mg9 (314-339), 1hj7 (314-393), 1hz8 (314-395), 1i0u (314-395), 1ijq (398-713), 1ldr (64-104), 2m7p (82-104), 2fcw (B=107-186), 2kri (B=147-186), 3m0c (C=4-788), 4ne9 (D=314-339), 2w2o (E=314-393), 2w2m (E=314-393), 3gcx (E=314-393), 3gcw (E=314-393), 2w2n (E=314-393), 2w2q (E=314-393), 2w2p (E=314-393), 3bps (E=314-393), 3p5c (L=276-715), 3p5b (L=316-715), 3so6 (Q=819-832)
OPM none
Complexes none
Interactions

CALX, Complex: LDLR:CALX, PubMed

VGFR1, Complex: VGFR1:LDLR, PubMed

Domains

AA: 25-63, PDBID: 1F5Y, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor domain class A

AA: 66-104, PDBID: 1F5Y, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor domain class A

AA: 107-143, PDBID: 1N7D, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor domain class A

AA: 146-184, PDBID: 1N7D, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor domain class A

AA: 195-231, PDBID: 1AJJ, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor domain class A

AA: 234-270, PDBID: 1D2J, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor domain class A

AA: 274-313, PDBID: 1N7D, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor domain class A

AA: 335-357, PDBID: 1HJ7, Subunit A, Seq Identity:100%, Complement Clr-like EGF-like

AA: 439-483, PDBID: 1IJQ, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor repeat class B

AA: 486-526, PDBID: 1IJQ, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor repeat class B

AA: 529-570, PDBID: 1IJQ, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor repeat class B

AA: 573-615, PDBID: 1IJQ, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor repeat class B

AA: 616-656, PDBID: 1IJQ, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor repeat class B

AA: 671-711, PDBID: 1IJQ, Subunit B, Seq Identity:100%, Coagulation Factor Xa inhibitory site

UniProt annotation for LDLR_HUMAN » Low-density lipoprotein receptor
FUNCTION: Binds LDL, the major cholesterol-carrying lipoprotein of plasma, and transports it into cells by endocytosis. In order to be internalized, the receptor-ligand complexes must first cluster into clathrin-coated pits. In case of HIV-1 infection, functions as a receptor for extracellular Tat in neurons, mediating its internalization in uninfected cells.

SUBUNIT: Interacts with LDLRAP1, ARRB1, SNX17. Interacts (via NPXY motif) with DAB2 (via PID domain); the interaction is impaired by tyrosine phosphorylation of the NPXY motif. Interacts with the full length immature form of PCSK9 (via C-terminus). Interacts with HCV E1/E2 heterodimer. Interacts with HIV-1 Tat.

DOMAIN: The NPXY motif mediates the interaction with the clathrin adaptor DAB2 involved in receptor internalization.

DISEASE: Familial hypercholesterolemia (FH) OMIM: Common autosomal semi-dominant disease that affects about 1 in 500 individuals. The receptor defect impairs the catabolism of LDL, and the resultant elevation in plasma LDL-cholesterol promotes deposition of cholesterol in the skin (xanthelasma), tendons (xanthomas), and coronary arteries (atherosclerosis). affecting the gene represented in this entry.

UniProt features for LDLR_HUMAN » Low-density lipoprotein receptor
SIGNAL 1 21
CHAIN 22 860 Low-density lipoprotein receptor.
DOMAIN 25 65 LDL-receptor class A 1.
DOMAIN 66 106 LDL-receptor class A 2.
DOMAIN 107 145 LDL-receptor class A 3.
DOMAIN 146 186 LDL-receptor class A 4.
DOMAIN 195 233 LDL-receptor class A 5.
DOMAIN 234 272 LDL-receptor class A 6.
DOMAIN 274 313 LDL-receptor class A 7.
DOMAIN 314 353 EGF-like 1.
DOMAIN 354 393 EGF-like 2; calcium-binding (Potential).
REPEAT 397 438 LDL-receptor class B 1.
REPEAT 439 485 LDL-receptor class B 2.
REPEAT 486 528 LDL-receptor class B 3.
REPEAT 529 572 LDL-receptor class B 4.
REPEAT 573 615 LDL-receptor class B 5.
REPEAT 616 658 LDL-receptor class B 6.
DOMAIN 663 712 EGF-like 3.
REGION 721 768 Clustered O-linked oligosaccharides.
REGION 811 860 Required for MYLIP-triggered down- regulation of LDLR.
MOTIF 823 828 NPXY motif.
DISULFID 27 39
DISULFID 34 52
DISULFID 46 63
DISULFID 68 82
DISULFID 75 95
DISULFID 89 104
DISULFID 109 121 By similarity.
DISULFID 116 134
DISULFID 128 143
DISULFID 148 160
DISULFID 155 173
DISULFID 167 184
DISULFID 197 209
DISULFID 204 222
DISULFID 216 231
DISULFID 236 248
DISULFID 243 261
DISULFID 255 270
DISULFID 276 289
DISULFID 284 302
DISULFID 296 313
DISULFID 318 329
DISULFID 325 338
DISULFID 340 352
DISULFID 358 368
DISULFID 364 377
DISULFID 379 392
DISULFID 667 681
DISULFID 677 696
DISULFID 698 711
Amino Acid Sequence for LDLR_HUMAN » Low-density lipoprotein receptor
MGPWGWKLRW TVALLLAAAG TAVGDRCERN EFQCQDGKCI SYKWVCDGSA ECQDGSDESQ ETCLSVTCKS GDFSCGGRVN RCIPQFWRCD GQVDCDNGSD EQGCPPKTCS QDEFRCHDGK CISRQFVCDS DRDCLDGSDE ASCPVLTCGP ASFQCNSSTC IPQLWACDND PDCEDGSDEW PQRCRGLYVF QGDSSPCSAF EFHCLSGECI HSSWRCDGGP DCKDKSDEEN CAVATCRPDE FQCSDGNCIH GSRQCDREYD CKDMSDEVGC VNVTLCEGPN KFKCHSGECI TLDKVCNMAR DCRDWSDEPI KECGTNECLD NNGGCSHVCN DLKIGYECLC PDGFQLVAQR RCEDIDECQD PDTCSQLCVN LEGGYKCQCE EGFQLDPHTK ACKAVGSIAY LFFTNRHEVR KMTLDRSEYT SLIPNLRNVV ALDTEVASNR IYWSDLSQRM ICSTQLDRAH GVSSYDTVIS RDIQAPDGLA VDWIHSNIYW TDSVLGTVSV ADTKGVKRKT LFRENGSKPR AIVVDPVHGF MYWTDWGTPA KIKKGGLNGV DIYSLVTENI QWPNGITLDL LSGRLYWVDS KLHSISSIDV NGGNRKTILE DEKRLAHPFS LAVFEDKVFW TDIINEAIFS ANRLTGSDVN LLAENLLSPE DMVLFHNLTQ PRGVNWCERT TLSNGGCQYL CLPAPQINPH SPKFTCACPD GMLLARDMRS CLTEAEAAVA TQETSTVRLK VSSTAVRTQH TTTRPVPDTS RLPGATPGLT TVEIVTMSHQ ALGDVAGRGN EKKPSSVRAL SIVLPIVLLV FLCLGVFLLW KNWRLKNINS INFDNPVYQK TTEDEVHICH NQDGYSYPSR QMVSLEDDVA