LRP2_HUMAN » Low-density lipoprotein receptor-related protein 2

LRP2_HUMAN » Low-density lipoprotein receptor-related protein 2
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
LRP2_HUMAN » Low-density lipoprotein receptor-related protein 2 » LRP-2; Glycoprotein 330;gp330; Megalin;
Hydrophobic Thickness 38.4 ± 1.2 Å
Tilt Angle 0 ± 2°
ΔGtransfer -52.5 kcal/mol
ΔGfold -28.0 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, Reactome, HMDB
Topology Out
TM Segments 4420-4448 (4420-4451)
Pathways

Hedgehog signaling pathway (KEGG)

Signal Transduction (Reactome)

Steroid hormones (Reactome)

PDB 2m0p (1103-1148)
OPM none
Complexes none
Interactions

A4, Complex: LRP2:A4, PubMed

APOA, Complex: LRP2:APOA, PubMed

MPRD, Complex: MPRD:LRP2

SYJ2B, Complex: SYJ2B:LRP2, PubMed

Domains

AA: 26-62, PDBID: 2I1P, Subunit A, Seq Identity:52%, Low-density lipoprotein receptor domain class A

AA: 65-103, PDBID: 1F5Y, Subunit A, Seq Identity:54%, Low-density lipoprotein receptor domain class A

AA: 106-142, PDBID: 2KNY, Subunit A, Seq Identity:53%, Low-density lipoprotein receptor domain class A

AA: 181-217, PDBID: 2KNY, Subunit A, Seq Identity:53%, Low-density lipoprotein receptor domain class A

AA: 220-256, PDBID: 2FYJ, Subunit A, Seq Identity:62%, Low-density lipoprotein receptor domain class A

AA: 264-301, PDBID: 1J8E, Subunit A, Seq Identity:44%, Low-density lipoprotein receptor domain class A

AA: 479-519, PDBID: 3S94, Subunit B, Seq Identity:47%, Low-density lipoprotein receptor repeat class B

AA: 522-566, PDBID: 3S94, Subunit B, Seq Identity:51%, Low-density lipoprotein receptor repeat class B

AA: 838-879, PDBID: 3S94, Subunit B, Seq Identity:55%, Low-density lipoprotein receptor repeat class B

AA: 1024-1060, PDBID: 2FYJ, Subunit A, Seq Identity:50%, Low-density lipoprotein receptor domain class A

AA: 1065-1101, PDBID: 1J8E, Subunit A, Seq Identity:56%, Low-density lipoprotein receptor domain class A

AA: 1107-1143, PDBID: 2M0P, Subunit A, Seq Identity:100%, Low-density lipoprotein receptor domain class A

AA: 1147-1183, PDBID: 2FYJ, Subunit A, Seq Identity:53%, Low-density lipoprotein receptor domain class A

AA: 1185-1222, PDBID: 2I1P, Subunit A, Seq Identity:72%, Low-density lipoprotein receptor domain class A

AA: 1228-1266, PDBID: 1CR8, Subunit A, Seq Identity:61%, Low-density lipoprotein receptor domain class A

AA: 1269-1305, PDBID: 2FYJ, Subunit A, Seq Identity:55%, Low-density lipoprotein receptor domain class A

AA: 1390-1428, PDBID: 1UZJ, Subunit B, Seq Identity:53%, Calcium-binding EGF domain

AA: 1521-1561, PDBID: 3S2K, Subunit A, Seq Identity:61%, Low-density lipoprotein receptor repeat class B

AA: 1566-1607, PDBID: 3V64, Subunit D, Seq Identity:56%, Low-density lipoprotein receptor repeat class B

AA: 1883-1928, PDBID: 3V64, Subunit D, Seq Identity:38%, Low-density lipoprotein receptor repeat class B

AA: 2022-2058, PDBID: 3S2K, Subunit A, Seq Identity:43%, Coagulation Factor Xa inhibitory site

AA: 2202-2243, PDBID: 3S2K, Subunit A, Seq Identity:47%, Low-density lipoprotein receptor repeat class B

AA: 2519-2560, PDBID: 3S94, Subunit B, Seq Identity:60%, Low-density lipoprotein receptor repeat class B

AA: 2563-2601, PDBID: 3S94, Subunit B, Seq Identity:42%, Low-density lipoprotein receptor repeat class B

AA: 2698-2736, PDBID: 1D2L, Subunit A, Seq Identity:58%, Low-density lipoprotein receptor domain class A

AA: 2739-2775, PDBID: 2FYJ, Subunit A, Seq Identity:65%, Low-density lipoprotein receptor domain class A

AA: 2778-2817, PDBID: 3T5O, Subunit A, Seq Identity:47%, Low-density lipoprotein receptor domain class A

AA: 2820-2859, PDBID: 1D2L, Subunit A, Seq Identity:56%, Low-density lipoprotein receptor domain class A

AA: 2862-2899, PDBID: 1J8E, Subunit A, Seq Identity:63%, Low-density lipoprotein receptor domain class A

AA: 2904-2943, PDBID: 1J8E, Subunit A, Seq Identity:57%, Low-density lipoprotein receptor domain class A

AA: 2946-2988, PDBID: 1F5Y, Subunit A, Seq Identity:48%, Low-density lipoprotein receptor domain class A

AA: 2991-3027, PDBID: 2FYJ, Subunit A, Seq Identity:55%, Low-density lipoprotein receptor domain class A

AA: 3030-3068, PDBID: 1D2L, Subunit A, Seq Identity:53%, Low-density lipoprotein receptor domain class A

AA: 3073-3109, PDBID: 2FYJ, Subunit A, Seq Identity:55%, Low-density lipoprotein receptor domain class A

AA: 3132-3155, PDBID: 3V65, Subunit D, Seq Identity:46%, Complement Clr-like EGF-like

AA: 3282-3330, PDBID: 3S2K, Subunit A, Seq Identity:38%, Low-density lipoprotein receptor repeat class B

AA: 3333-3374, PDBID: 3S94, Subunit B, Seq Identity:51%, Low-density lipoprotein receptor repeat class B

AA: 3377-3417, PDBID: 3S94, Subunit B, Seq Identity:57%, Low-density lipoprotein receptor repeat class B

AA: 3510-3548, PDBID: 1J8E, Subunit A, Seq Identity:55%, Low-density lipoprotein receptor domain class A

AA: 3551-3589, PDBID: 1D2J, Subunit A, Seq Identity:40%, Low-density lipoprotein receptor domain class A

AA: 3592-3630, PDBID: 1J8E, Subunit A, Seq Identity:53%, Low-density lipoprotein receptor domain class A

AA: 3633-3671, PDBID: 2FYJ, Subunit A, Seq Identity:65%, Low-density lipoprotein receptor domain class A

AA: 3676-3714, PDBID: 2I1P, Subunit A, Seq Identity:50%, Low-density lipoprotein receptor domain class A

AA: 3717-3754, PDBID: 2FYJ, Subunit A, Seq Identity:57%, Low-density lipoprotein receptor domain class A

AA: 3757-3793, PDBID: 3T5O, Subunit A, Seq Identity:53%, Low-density lipoprotein receptor domain class A

AA: 3796-3832, PDBID: 2FYJ, Subunit A, Seq Identity:51%, Low-density lipoprotein receptor domain class A

AA: 3840-3878, PDBID: 1D2L, Subunit A, Seq Identity:52%, Low-density lipoprotein receptor domain class A

AA: 3881-3920, PDBID: 3T5O, Subunit A, Seq Identity:50%, Low-density lipoprotein receptor domain class A

AA: 3926-3962, PDBID: 2M0P, Subunit A, Seq Identity:48%, Low-density lipoprotein receptor domain class A

AA: 4007-4047, PDBID: 1Z6C, Subunit A, Seq Identity:40%, Calcium-binding EGF domain

AA: 4154-4194, PDBID: 3V64, Subunit D, Seq Identity:51%, Low-density lipoprotein receptor repeat class B

AA: 4197-4238, PDBID: 3S94, Subunit B, Seq Identity:60%, Low-density lipoprotein receptor repeat class B

UniProt annotation for LRP2_HUMAN » Low-density lipoprotein receptor-related protein 2
FUNCTION: Acts together with cubilin to mediate HDL endocytosis (By similarity). May participate in regulation of parathyroid- hormone and para-thyroid-hormone-related protein release.

SUBUNIT: Binds plasminogen, extracellular matrix components, plasminogen activator-plasminogen activator inhibitor type I complex, apolipoprotein E-enriched beta-VLDL, lipoprotein lipase, lactoferrin, CLU/clusterin and calcium. Forms a multimeric complex together with a receptor-associated protein (RAP). Binds to ankyrin-repeat family A protein 2 (ANKRA2). Interacts with LRP2BP. Interacts (via NPXY motif) with DAB2; the interaction is not affected by tyrosine phosphorylation of the NPXY motif.

TISSUE SPECIFICITY: Absorptive epithelia, including renal proximal tubules.

DISEASE: Donnai-Barrow syndrome (DBS) OMIM: Rare autosomal recessive disorder characterized by major malformations including agenesis of the corpus callosum, congenital diaphragmatic hernia, facial dysmorphology, ocular anomalies, sensorineural hearing loss and developmental delay. The FOAR syndrome was first described as comprising facial anomalies, ocular anomalies, sensorineural hearing loss, and proteinuria. DBS and FOAR were first described as distinct disorders but the classic distinguishing features between the 2 disorders were presence of proteinuria and absence of diaphragmatic hernia and corpus callosum anomalies in FOAR. Early reports noted that the 2 disorders shared many phenotypic features and may be identical. Although there is variability in the expression of some features (e.g., agenesis of the corpus callosum and proteinuria), DBS and FOAR are now considered to represent the same entity. disease is caused by mutations affecting the gene represented in this entry.

Amino Acid Sequence for LRP2_HUMAN » Low-density lipoprotein receptor-related protein 2
MDRGPAAVAC TLLLALVACL APASGQECDS AHFRCGSGHC IPADWRCDGT KDCSDDADEI GCAVVTCQQG YFKCQSEGQC IPNSWVCDQD QDCDDGSDER QDCSQSTCSS HQITCSNGQC IPSEYRCDHV RDCPDGADEN DCQYPTCEQL TCDNGACYNT SQKCDWKVDC RDSSDEINCT EICLHNEFSC GNGECIPRAY VCDHDNDCQD GSDEHACNYP TCGGYQFTCP SGRCIYQNWV CDGEDDCKDN GDEDGCESGP HDVHKCSPRE WSCPESGRCI SIYKVCDGIL DCPGREDENN TSTGKYCSMT LCSALNCQYQ CHETPYGGAC FCPPGYIINH NDSRTCVEFD DCQIWGICDQ KCESRPGRHL CHCEEGYILE RGQYCKANDS FGEASIIFSN GRDLLIGDIH GRSFRILVES QNRGVAVGVA FHYHLQRVFW TDTVQNKVFS VDINGLNIQE VLNVSVETPE NLAVDWVNNK IYLVETKVNR IDMVNLDGSY RVTLITENLG HPRGIAVDPT VGYLFFSDWE SLSGEPKLER AFMDGSNRKD LVKTKLGWPA GVTLDMISKR VYWVDSRFDY IETVTYDGIQ RKTVVHGGSL IPHPFGVSLF EGQVFFTDWT KMAVLKANKF TETNPQVYYQ ASLRPYGVTV YHSLRQPYAT NPCKDNNGGC EQVCVLSHRT DNDGLGFRCK CTFGFQLDTD ERHCIAVQNF LIFSSQVAIR GIPFTLSTQE DVMVPVSGNP SFFVGIDFDA QDSTIFFSDM SKHMIFKQKI DGTGREILAA NRVENVESLA FDWISKNLYW TDSHYKSISV MRLADKTRRT VVQYLNNPRS VVVHPFAGYL FFTDWFRPAK IMRAWSDGSH LLPVINTTLG WPNGLAIDWA ASRLYWVDAY FDKIEHSTFD GLDRRRLGHI EQMTHPFGLA IFGEHLFFTD WRLGAIIRVR KADGGEMTVI RSGIAYILHL KSYDVNIQTG SNACNQPTHP NGDCSHFCFP VPNFQRVCGC PYGMRLASNH LTCEGDPTNE PPTEQCGLFS FPCKNGRCVP NYYLCDGVDD CHDNSDEQLC GTLNNTCSSS AFTCGHGECI PAHWRCDKRN DCVDGSDEHN CPTHAPASCL DTQYTCDNHQ CISKNWVCDT DNDCGDGSDE KNCNSTETCQ PSQFNCPNHR CIDLSFVCDG DKDCVDGSDE VGCVLNCTAS QFKCASGDKC IGVTNRCDGV FDCSDNSDEA GCPTRPPGMC HSDEFQCQED GICIPNFWEC DGHPDCLYGS DEHNACVPKT CPSSYFHCDN GNCIHRAWLC DRDNDCGDMS DEKDCPTQPF RCPSWQWQCL GHNICVNLSV VCDGIFDCPN GTDESPLCNG NSCSDFNGGC THECVQEPFG AKCLCPLGFL LANDSKTCED IDECDILGSC SQHCYNMRGS FRCSCDTGYM LESDGRTCKV TASESLLLLV ASQNKIIADS VTSQVHNIYS LVENGSYIVA VDFDSISGRI FWSDATQGKT WSAFQNGTDR RVVFDSSIIL TETIAIDWVG RNLYWTDYAL ETIEVSKIDG SHRTVLISKN LTNPRGLALD PRMNEHLLFW SDWGHHPRIE RASMDGSMRT VIVQDKIFWP CGLTIDYPNR LLYFMDSYLD YMDFCDYNGH HRRQVIASDL IIRHPYALTL FEDSVYWTDR ATRRVMRANK WHGGNQSVVM YNIQWPLGIV AVHPSKQPNS VNPCAFSRCS HLCLLSSQGP HFYSCVCPSG WSLSPDLLNC LRDDQPFLIT VRQHIIFGIS LNPEVKSNDA MVPIAGIQNG LDVEFDDAEQ YIYWVENPGE IHRVKTDGTN RTVFASISMV GPSMNLALDW ISRNLYSTNP RTQSIEVLTL HGDIRYRKTL IANDGTALGV GFPIGITVDP ARGKLYWSDQ GTDSGVPAKI ASANMDGTSV KTLFTGNLEH LECVTLDIEE QKLYWAVTGR GVIERGNVDG TDRMILVHQL SHPWGIAVHD SFLYYTDEQY EVIERVDKAT GANKIVLRDN VPNLRGLQVY HRRNAAESSN GCSNNMNACQ QICLPVPGGL FSCACATGFK LNPDNRSCSP YNSFIVVSML SAIRGFSLEL SDHSETMVPV AGQGRNALHV DVDVSSGFIY WCDFSSSVAS DNAIRRIKPD GSSLMNIVTH GIGENGVRGI AVDWVAGNLY FTNAFVSETL IEVLRINTTY RRVLLKVTVD MPRHIVVDPK NRYLFWADYG QRPKIERSFL DCTNRTVLVS EGIVTPRGLA VDRSDGYVYW VDDSLDIIAR IRINGENSEV IRYGSRYPTP YGITVFENSI IWVDRNLKKI FQASKEPENT EPPTVIRDNI NWLRDVTIFD KQVQPRSPAE VNNNPCLENN GGCSHLCFAL PGLHTPKCDC AFGTLQSDGK NCAISTENFL IFALSNSLRS LHLDPENHSP PFQTINVERT VMSLDYDSVS DRIYFTQNLA SGVGQISYAT LSSGIHTPTV IASGIGTADG IAFDWITRRI YYSDYLNQMI NSMAEDGSNR TVIARVPKPR AIVLDPCQGY LYWADWDTHA KIERATLGGN FRVPIVNSSL VMPSGLTLDY EEDLLYWVDA SLQRIERSTL TGVDREVIVN AAVHAFGLTL YGQYIYWTDL YTQRIYRANK YDGSGQIAMT TNLLSQPRGI NTVVKNQKQQ CNNPCEQFNG GCSHICAPGP NGAECQCPHE GNWYLANNRK HCIVDNGERC GASSFTCSNG RCISEEWKCD NDNDCGDGSD EMESVCALHT CSPTAFTCAN GRCVQYSYRC DYYNDCGDGS DEAGCLFRDC NATTEFMCNN RRCIPREFIC NGVDNCHDNN TSDEKNCPDR TCQSGYTKCH NSNICIPRVY LCDGDNDCGD NSDENPTYCT THTCSSSEFQ CASGRCIPQH WYCDQETDCF DASDEPASCG HSERTCLADE FKCDGGRCIP SEWICDGDND CGDMSDEDKR HQCQNQNCSD SEFLCVNDRP PDRRCIPQSW VCDGDVDCTD GYDENQNCTR RTCSENEFTC GYGLCIPKIF RCDRHNDCGD YSDERGCLYQ TCQQNQFTCQ NGRCISKTFV CDEDNDCGDG SDELMHLCHT PEPTCPPHEF KCDNGRCIEM MKLCNHLDDC LDNSDEKGCG INECHDPSIS GCDHNCTDTL TSFYCSCRPG YKLMSDKRTC VDIDECTEMP FVCSQKCENV IGSYICKCAP GYLREPDGKT CRQNSNIEPY LIFSNRYYLR NLTIDGYFYS LILEGLDNVV ALDFDRVEKR LYWIDTQRQV IERMFLNKTN KETIINHRLP AAESLAVDWV SRKLYWLDAR LDGLFVSDLN GGHRRMLAQH CVDANNTFCF DNPRGLALHP QYGYLYWADW GHRAYIGRVG MDGTNKSVII STKLEWPNGI TIDYTNDLLY WADAHLGYIE YSDLEGHHRH TVYDGALPHP FAITIFEDTI YWTDWNTRTV EKGNKYDGSN RQTLVNTTHR PFDIHVYHPY RQPIVSNPCG TNNGGCSHLC LIKPGGKGFT CECPDDFRTL QLSGSTYCMP MCSSTQFLCA NNEKCIPIWW KCDGQKDCSD GSDELALCPQ RFCRLGQFQC SDGNCTSPQT LCNAHQNCPD GSDEDRLLCE NHHCDSNEWQ CANKRCIPES WQCDTFNDCE DNSDEDSSHC ASRTCRPGQF RCANGRCIPQ AWKCDVDNDC GDHSDEPIEE CMSSAHLCDN FTEFSCKTNY RCIPKWAVCN GVDDCRDNSD EQGCEERTCH PVGDFRCKNH HCIPLRWQCD GQNDCGDNSD EENCAPRECT ESEFRCVNQQ CIPSRWICDH YNDCGDNSDE RDCEMRTCHP EYFQCTSGHC VHSELKCDGS ADCLDASDEA DCPTRFPDGA YCQATMFECK NHVCIPPYWK CDGDDDCGDG SDEELHLCLD VPCNSPNRFR CDNNRCIYSH EVCNGVDDCG DGTDETEEHC RKPTPKPCTE YEYKCGNGHC IPHDNVCDDA DDCGDWSDEL GCNKGKERTC AENICEQNCT QLNEGGFICS CTAGFETNVF DRTSCLDINE CEQFGTCPQH CRNTKGSYEC VCADGFTSMS DRPGKRCAAE GSSPLLLLPD NVRIRKYNLS SERFSEYLQD EEYIQAVDYD WDPKDIGLSV VYYTVRGEGS RFGAIKRAYI PNFESGRNNL VQEVDLKLKY VMQPDGIAVD WVGRHIYWSD VKNKRIEVAK LDGRYRKWLI STDLDQPAAI AVNPKLGLMF WTDWGKEPKI ESAWMNGEDR NILVFEDLGW PTGLSIDYLN NDRIYWSDFK EDVIETIKYD GTDRRVIAKE AMNPYSLDIF EDQLYWISKE KGEVWKQNKF GQGKKEKTLV VNPWLTQVRI FHQLRYNKSV PNLCKQICSH LCLLRPGGYS CACPQGSSFI EGSTTECDAA IELPINLPPP CRCMHGGNCY FDETDLPKCK CPSGYTGKYC EMAFSKGISP GTTAVAVLLT ILLIVVIGAL AIAGFFHYRR TGSLLPALPK LPSLSSLVKP SENGNGVTFR SGADLNMDIG VSGFGPETAI DRSMAMSEDF VMEMGKQPII FENPMYSARD SAVKVVQPIQ VTVSENVDNK NYGSPINPSE IVPETNPTSP AADGTQVTKW NLFKRKSKQT TNFENPIYAQ MENEQKESVA ATPPPSPSLP AKPKPPSRRD PTPTYSATED TFKDTANLVK EDSEV