Uniprot ID:P98164	Protein name: Low-density lipoprotein receptor-related protein 2	Gene : LRP2
Protein Family:LDLR family	Squence Length : 4655

Sequnce	>P98164 4656 MDRGPAAVACTLLLALVACLAPASGQECDSAHFRCGSGHCIPADWRCDGTKDCSDDADEIGCAVVTCQQGYFKCQSEGQCIPNSWVCDQDQDCDDGSDERQDCSQSTCSSHQITCSNGQCIPSEYRCDHVRDCPDGADENDCQYPTCEQLTCDNGACYNTSQKCDWKVDCRDSSDEINCTEICLHNEFSCGNGECIPRAYVCDHDNDCQDGSDEHACNYPTCGGYQFTCPSGRCIYQNWVCDGEDDCKDNGDEDGCESGPHDVHKCSPREWSCPESGRCISIYKVCDGILDCPGREDENNTSTGKYCSMTLCSALNCQYQCHETPYGGACFCPPGYIINHNDSRTCVEFDDCQIWGICDQKCESRPGRHLCHCEEGYILERGQYCKANDSFGEASIIFSNGRDLLIGDIHGRSFRILVESQNRGVAVGVAFHYHLQRVFWTDTVQNKVFSVDINGLNIQEVLNVSVETPENLAVDWVNNKIYLVETKVNRIDMVNLDGSYRVTLITENLGHPRGIAVDPTVGYLFFSDWESLSGEPKLERAFMDGSNRKDLVKTKLGWPAGVTLDMISKRVYWVDSRFDYIETVTYDGIQRKTVVHGGSLIPHPFGVSLFEGQVFFTDWTKMAVLKANKFTETNPQVYYQASLRPYGVTVYHSLRQPYATNPCKDNNGGCEQVCVLSHRTDNDGLGFRCKCTFGFQLDTDERHCIAVQNFLIFSSQVAIRGIPFTLSTQEDVMVPVSGNPSFFVGIDFDAQDSTIFFSDMSKHMIFKQKIDGTGREILAANRVENVESLAFDWISKNLYWTDSHYKSISVMRLADKTRRTVVQYLNNPRSVVVHPFAGYLFFTDWFRPAKIMRAWSDGSHLLPVINTTLGWPNGLAIDWAASRLYWVDAYFDKIEHSTFDGLDRRRLGHIEQMTHPFGLAIFGEHLFFTDWRLGAIIRVRKADGGEMTVIRSGIAYILHLKSYDVNIQTGSNACNQPTHPNGDCSHFCFPVPNFQRVCGCPYGMRLASNHLTCEGDPTNEPPTEQCGLFSFPCKNGRCVPNYYLCDGVDDCHDNSDEQLCGTLNNTCSSSAFTCGHGECIPAHWRCDKRNDCVDGSDEHNCPTHAPASCLDTQYTCDNHQCISKNWVCDTDNDCGDGSDEKNCNSTETCQPSQFNCPNHRCIDLSFVCDGDKDCVDGSDEVGCVLNCTASQFKCASGDKCIGVTNRCDGVFDCSDNSDEAGCPTRPPGMCHSDEFQCQEDGICIPNFWECDGHPDCLYGSDEHNACVPKTCPSSYFHCDNGNCIHRAWLCDRDNDCGDMSDEKDCPTQPFRCPSWQWQCLGHNICVNLSVVCDGIFDCPNGTDESPLCNGNSCSDFNGGCTHECVQEPFGAKCLCPLGFLLANDSKTCEDIDECDILGSCSQHCYNMRGSFRCSCDTGYMLESDGRTCKVTASESLLLLVASQNKIIADSVTSQVHNIYSLVENGSYIVAVDFDSISGRIFWSDATQGKTWSAFQNGTDRRVVFDSSIILTETIAIDWVGRNLYWTDYALETIEVSKIDGSHRTVLISKNLTNPRGLALDPRMNEHLLFWSDWGHHPRIERASMDGSMRTVIVQDKIFWPCGLTIDYPNRLLYFMDSYLDYMDFCDYNGHHRRQVIASDLIIRHPYALTLFEDSVYWTDRATRRVMRANKWHGGNQSVVMYNIQWPLGIVAVHPSKQPNSVNPCAFSRCSHLCLLSSQGPHFYSCVCPSGWSLSPDLLNCLRDDQPFLITVRQHIIFGISLNPEVKSNDAMVPIAGIQNGLDVEFDDAEQYIYWVENPGEIHRVKTDGTNRTVFASISMVGPSMNLALDWISRNLYSTNPRTQSIEVLTLHGDIRYRKTLIANDGTALGVGFPIGITVDPARGKLYWSDQGTDSGVPAKIASANMDGTSVKTLFTGNLEHLECVTLDIEEQKLYWAVTGRGVIERGNVDGTDRMILVHQLSHPWGIAVHDSFLYYTDEQYEVIERVDKATGANKIVLRDNVPNLRGLQVYHRRNAAESSNGCSNNMNACQQICLPVPGGLFSCACATGFKLNPDNRSCSPYNSFIVVSMLSAIRGFSLELSDHSETMVPVAGQGRNALHVDVDVSSGFIYWCDFSSSVASDNAIRRIKPDGSSLMNIVTHGIGENGVRGIAVDWVAGNLYFTNAFVSETLIEVLRINTTYRRVLLKVTVDMPRHIVVDPKNRYLFWADYGQRPKIERSFLDCTNRTVLVSEGIVTPRGLAVDRSDGYVYWVDDSLDIIARIRINGENSEVIRYGSRYPTPYGITVFENSIIWVDRNLKKIFQASKEPENTEPPTVIRDNINWLRDVTIFDKQVQPRSPAEVNNNPCLENNGGCSHLCFALPGLHTPKCDCAFGTLQSDGKNCAISTENFLIFALSNSLRSLHLDPENHSPPFQTINVERTVMSLDYDSVSDRIYFTQNLASGVGQISYATLSSGIHTPTVIASGIGTADGIAFDWITRRIYYSDYLNQMINSMAEDGSNRTVIARVPKPRAIVLDPCQGYLYWADWDTHAKIERATLGGNFRVPIVNSSLVMPSGLTLDYEEDLLYWVDASLQRIERSTLTGVDREVIVNAAVHAFGLTLYGQYIYWTDLYTQRIYRANKYDGSGQIAMTTNLLSQPRGINTVVKNQKQQCNNPCEQFNGGCSHICAPGPNGAECQCPHEGNWYLANNRKHCIVDNGERCGASSFTCSNGRCISEEWKCDNDNDCGDGSDEMESVCALHTCSPTAFTCANGRCVQYSYRCDYYNDCGDGSDEAGCLFRDCNATTEFMCNNRRCIPREFICNGVDNCHDNNTSDEKNCPDRTCQSGYTKCHNSNICIPRVYLCDGDNDCGDNSDENPTYCTTHTCSSSEFQCASGRCIPQHWYCDQETDCFDASDEPASCGHSERTCLADEFKCDGGRCIPSEWICDGDNDCGDMSDEDKRHQCQNQNCSDSEFLCVNDRPPDRRCIPQSWVCDGDVDCTDGYDENQNCTRRTCSENEFTCGYGLCIPKIFRCDRHNDCGDYSDERGCLYQTCQQNQFTCQNGRCISKTFVCDEDNDCGDGSDELMHLCHTPEPTCPPHEFKCDNGRCIEMMKLCNHLDDCLDNSDEKGCGINECHDPSISGCDHNCTDTLTSFYCSCRPGYKLMSDKRTCVDIDECTEMPFVCSQKCENVIGSYICKCAPGYLREPDGKTCRQNSNIEPYLIFSNRYYLRNLTIDGYFYSLILEGLDNVVALDFDRVEKRLYWIDTQRQVIERMFLNKTNKETIINHRLPAAESLAVDWVSRKLYWLDARLDGLFVSDLNGGHRRMLAQHCVDANNTFCFDNPRGLALHPQYGYLYWADWGHRAYIGRVGMDGTNKSVIISTKLEWPNGITIDYTNDLLYWADAHLGYIEYSDLEGHHRHTVYDGALPHPFAITIFEDTIYWTDWNTRTVEKGNKYDGSNRQTLVNTTHRPFDIHVYHPYRQPIVSNPCGTNNGGCSHLCLIKPGGKGFTCECPDDFRTLQLSGSTYCMPMCSSTQFLCANNEKCIPIWWKCDGQKDCSDGSDELALCPQRFCRLGQFQCSDGNCTSPQTLCNAHQNCPDGSDEDRLLCENHHCDSNEWQCANKRCIPESWQCDTFNDCEDNSDEDSSHCASRTCRPGQFRCANGRCIPQAWKCDVDNDCGDHSDEPIEECMSSAHLCDNFTEFSCKTNYRCIPKWAVCNGVDDCRDNSDEQGCEERTCHPVGDFRCKNHHCIPLRWQCDGQNDCGDNSDEENCAPRECTESEFRCVNQQCIPSRWICDHYNDCGDNSDERDCEMRTCHPEYFQCTSGHCVHSELKCDGSADCLDASDEADCPTRFPDGAYCQATMFECKNHVCIPPYWKCDGDDDCGDGSDEELHLCLDVPCNSPNRFRCDNNRCIYSHEVCNGVDDCGDGTDETEEHCRKPTPKPCTEYEYKCGNGHCIPHDNVCDDADDCGDWSDELGCNKGKERTCAENICEQNCTQLNEGGFICSCTAGFETNVFDRTSCLDINECEQFGTCPQHCRNTKGSYECVCADGFTSMSDRPGKRCAAEGSSPLLLLPDNVRIRKYNLSSERFSEYLQDEEYIQAVDYDWDPKDIGLSVVYYTVRGEGSRFGAIKRAYIPNFESGRNNLVQEVDLKLKYVMQPDGIAVDWVGRHIYWSDVKNKRIEVAKLDGRYRKWLISTDLDQPAAIAVNPKLGLMFWTDWGKEPKIESAWMNGEDRNILVFEDLGWPTGLSIDYLNNDRIYWSDFKEDVIETIKYDGTDRRVIAKEAMNPYSLDIFEDQLYWISKEKGEVWKQNKFGQGKKEKTLVVNPWLTQVRIFHQLRYNKSVPNLCKQICSHLCLLRPGGYSCACPQGSSFIEGSTTECDAAIELPINLPPPCRCMHGGNCYFDETDLPKCKCPSGYTGKYCEMAFSKGISPGTTAVAVLLTILLIVVIGALAIAGFFHYRRTGSLLPALPKLPSLSSLVKPSENGNGVTFRSGADLNMDIGVSGFGPETAIDRSMAMSEDFVMEMGKQPIIFENPMYSARDSAVKVVQPIQVTVSENVDNKNYGSPINPSEIVPETNPTSPAADGTQVTKWNLFKRKSKQTTNFENPIYAQMENEQKESVAATPPPSPSLPAKPKPPSRRDPTPTYSATEDTFKDTANLVKEDSEV
Domains	DOMAIN 27 63 LDL-receptor class A 1 DOMAIN 66 104 LDL-receptor class A 2 DOMAIN 107 143 LDL-receptor class A 3 DOMAIN 146 180 LDL-receptor class A 4 DOMAIN 182 218 LDL-receptor class A 5 DOMAIN 221 257 LDL-receptor class A 6 DOMAIN 265 308 LDL-receptor class A 7 DOMAIN 1025 1061 LDL-receptor class A 8 DOMAIN 1066 1102 LDL-receptor class A 9 DOMAIN 1108 1144 LDL-receptor class A 10 DOMAIN 1148 1184 LDL-receptor class A 11 DOMAIN 1186 1223 LDL-receptor class A 12 DOMAIN 1229 1267 LDL-receptor class A 13 DOMAIN 1270 1306 LDL-receptor class A 14 DOMAIN 1304 1349 LDL-receptor class A 15 DOMAIN 1390 1429 EGF-like 1 calcium-binding DOMAIN 1700 1741 EGF-like 2 DOMAIN 2699 2737 LDL-receptor class A 16 DOMAIN 2740 2776 LDL-receptor class A 17 DOMAIN 2779 2818 LDL-receptor class A 18 DOMAIN 2821 2860 LDL-receptor class A 19 DOMAIN 2863 2900 LDL-receptor class A 20 DOMAIN 2905 2944 LDL-receptor class A 21 DOMAIN 2947 2989 LDL-receptor class A 22 DOMAIN 2992 3028 LDL-receptor class A 23 DOMAIN 3031 3069 LDL-receptor class A 24 DOMAIN 3074 3110 LDL-receptor class A 25 DOMAIN 3110 3151 EGF-like 3 DOMAIN 3152 3192 EGF-like 4 calcium-binding DOMAIN 3511 3549 LDL-receptor class A 26 DOMAIN 3552 3590 LDL-receptor class A 27 DOMAIN 3593 3631 LDL-receptor class A 28 DOMAIN 3634 3672 LDL-receptor class A 29 DOMAIN 3677 3715 LDL-receptor class A 30 DOMAIN 3718 3755 LDL-receptor class A 31 DOMAIN 3758 3794 LDL-receptor class A 32 DOMAIN 3797 3833 LDL-receptor class A 33 DOMAIN 3841 3879 LDL-receptor class A 34 DOMAIN 3882 3921 LDL-receptor class A 35 DOMAIN 3927 3963 LDL-receptor class A 36 DOMAIN 4007 4048 EGF-like 5 calcium-binding DOMAIN 4377 4411 EGF-like 6
LDL-receptor class A sequence regions	27 - 63 DSAHFRCGSGHCIPADWRCDGTKDCSDDADEIGCAVV 66 - 104 QQGYFKCQSEGQCIPNSWVCDQDQDCDDGSDERQDCSQS 107 - 143 SSHQITCSNGQCIPSEYRCDHVRDCPDGADENDCQYP 146 - 180 EQLTCDNGACYNTSQKCDWKVDCRDSSDEINCTEI 182 - 218 LHNEFSCGNGECIPRAYVCDHDNDCQDGSDEHACNYP 221 - 257 GGYQFTCPSGRCIYQNWVCDGEDDCKDNGDEDGCESG 265 - 308 SPREWSCPESGRCISIYKVCDGILDCPGREDENNTSTGKYCSMT 1025 - 1061 GLFSFPCKNGRCVPNYYLCDGVDDCHDNSDEQLCGTL 1066 - 1102 SSSAFTCGHGECIPAHWRCDKRNDCVDGSDEHNCPTH 1108 - 1144 LDTQYTCDNHQCISKNWVCDTDNDCGDGSDEKNCNST 1148 - 1184 QPSQFNCPNHRCIDLSFVCDGDKDCVDGSDEVGCVLN 1186 - 1223 TASQFKCASGDKCIGVTNRCDGVFDCSDNSDEAGCPTR 1229 - 1267 HSDEFQCQEDGICIPNFWECDGHPDCLYGSDEHNACVPK 1270 - 1306 PSSYFHCDNGNCIHRAWLCDRDNDCGDMSDEKDCPTQ 1304 - 1349 PTQPFRCPSWQWQCLGHNICVNLSVVCDGIFDCPNGTDESPLCNGN 2699 - 2737 GASSFTCSNGRCISEEWKCDNDNDCGDGSDEMESVCALH 2740 - 2776 SPTAFTCANGRCVQYSYRCDYYNDCGDGSDEAGCLFR 2779 - 2818 NATTEFMCNNRRCIPREFICNGVDNCHDNNTSDEKNCPDR 2821 - 2860 QSGYTKCHNSNICIPRVYLCDGDNDCGDNSDENPTYCTTH 2863 - 2900 SSSEFQCASGRCIPQHWYCDQETDCFDASDEPASCGHS 2905 - 2944 LADEFKCDGGRCIPSEWICDGDNDCGDMSDEDKRHQCQNQ 2947 - 2989 SDSEFLCVNDRPPDRRCIPQSWVCDGDVDCTDGYDENQNCTRR 2992 - 3028 SENEFTCGYGLCIPKIFRCDRHNDCGDYSDERGCLYQ 3031 - 3069 QQNQFTCQNGRCISKTFVCDEDNDCGDGSDELMHLCHTP 3074 - 3110 PPHEFKCDNGRCIEMMKLCNHLDDCLDNSDEKGCGIN 3511 - 3549 SSTQFLCANNEKCIPIWWKCDGQKDCSDGSDELALCPQR 3552 - 3590 RLGQFQCSDGNCTSPQTLCNAHQNCPDGSDEDRLLCENH 3593 - 3631 DSNEWQCANKRCIPESWQCDTFNDCEDNSDEDSSHCASR 3634 - 3672 RPGQFRCANGRCIPQAWKCDVDNDCGDHSDEPIEECMSS 3677 - 3715 DNFTEFSCKTNYRCIPKWAVCNGVDDCRDNSDEQGCEER 3718 - 3755 HPVGDFRCKNHHCIPLRWQCDGQNDCGDNSDEENCAPR 3758 - 3794 TESEFRCVNQQCIPSRWICDHYNDCGDNSDERDCEMR 3797 - 3833 HPEYFQCTSGHCVHSELKCDGSADCLDASDEADCPTR 3841 - 3879 QATMFECKNHVCIPPYWKCDGDDDCGDGSDEELHLCLDV 3882 - 3921 NSPNRFRCDNNRCIYSHEVCNGVDDCGDGTDETEEHCRKP 3927 - 3963 TEYEYKCGNGHCIPHDNVCDDADDCGDWSDELGCNKG
Function	"Multiligand endocytic receptor (By similarity). Acts together with CUBN to mediate endocytosis of high-density lipoproteins (By similarity). Mediates receptor-mediated uptake of polybasic drugs such as aprotinin, aminoglycosides and polymyxin B (By similarity). In the kidney, mediates the tubular uptake and clearance of leptin (By similarity). Also mediates transport of leptin across the blood-brain barrier through endocytosis at the choroid plexus epithelium (By similarity). Endocytosis of leptin in neuronal cells is required for hypothalamic leptin signaling and leptin-mediated regulation of feeding and body weight (By similarity). Mediates endocytosis and subsequent lysosomal degradation of CST3 in kidney proximal tubule cells (By similarity). Mediates renal uptake of 25-hydroxyvitamin D3 in complex with the vitamin D3 transporter GC/DBP (By similarity). Mediates renal uptake of metallothionein-bound heavy metals (PubMed:15126248). Together with CUBN, mediates renal reabsorption of myoglobin (By similarity). Mediates renal uptake and subsequent lysosomal degradation of APOM (By similarity). Plays a role in kidney selenium homeostasis by mediating renal endocytosis of selenoprotein SEPP1 (By similarity). Mediates renal uptake of the antiapoptotic protein BIRC5/survivin which may be important for functional integrity of the kidney (PubMed:23825075). Mediates renal uptake of matrix metalloproteinase MMP2 in complex with metalloproteinase inhibitor TIMP1 (By similarity). Mediates endocytosis of Sonic hedgehog protein N-product (ShhN), the active product of SHH (By similarity). Also mediates ShhN transcytosis (By similarity). In the embryonic neuroepithelium, mediates endocytic uptake and degradation of BMP4, is required for correct SHH localization in the ventral neural tube and plays a role in patterning of the ventral telencephalon (By similarity). Required at the onset of neurulation to sequester SHH on the apical surface of neuroepithelial cells of the rostral diencephalon ventral midline and to control PTCH1-dependent uptake and intracellular trafficking of SHH (By similarity). During neurulation, required in neuroepithelial cells for uptake of folate bound to the folate receptor FOLR1 which is necessary for neural tube closure (By similarity). In the adult brain, negatively regulates BMP signaling in the subependymal zone which enables neurogenesis to proceed (By similarity). In astrocytes, mediates endocytosis of ALB which is required for the synthesis of the neurotrophic factor oleic acid (By similarity). Involved in neurite branching (By similarity). During optic nerve development, required for SHH-mediated migration and proliferation of oligodendrocyte precursor cells (By similarity). Mediates endocytic uptake and clearance of SHH in the retinal margin which protects retinal progenitor cells from mitogenic stimuli and keeps them quiescent (By similarity). Plays a role in reproductive organ development by mediating uptake in reproductive tissues of androgen and estrogen bound to the sex hormone binding protein SHBG (By similarity). Mediates endocytosis of angiotensin-2 (By similarity). Also mediates endocytosis of angiotensis 1-7 (By similarity). Binds to the complex composed of beta-amyloid protein 40 and CLU/APOJ and mediates its endocytosis and lysosomal degradation (By similarity). Required for embryonic heart development (By similarity). Required for normal hearing, possibly through interaction with estrogen in the inner ear (By similarity)"
Motifs	MOTIF 4453 4462 SH3-binding. MOTIF 4456 4461 PxLPxI/L motif 1. mediates interaction with ANKRA2. MOTIF 4459 4464 PxLPxI/L motif 2. mediates interaction with ANKRA2. MOTIF 4521 4526 Endocytosis signal. MOTIF 4595 4598 NPXY motif. MOTIF 4598 4601 SH2-binding. MOTIF 4611 4622 SH3-binding
Disease	"DISEASE: Donnai-Barrow syndrome (DBS) [MIM:222448]: 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 Note=The disease is caused by mutations affecting the gene represented in this entry"

P98164 Proteins LDL-receptor class A domain Repeats