Description
| Product Name: | Human LRP4 Recombinant Protein |
| Product Code: | RPPB3877 |
| Size: | 10µg |
| Species: | Human |
| Target: | LRP4 |
| Synonyms: | Low-density lipoprotein receptor-related protein 4, LRP-4, Multiple epidermal growth factor-like domains 7, LRP4, KIAA0816, LRP10, MEGF7. |
| Source: | HEK293 Cells |
| Physical Appearance: | Filtered White lyophilized (freeze-dried) powder. |
| Formulation: | LRP4 filtered (0.4 �m) and lyophilized from 0.5mg/ml solution in PBS, 5% (w/v) trehalose pH 7.5. |
| Solubility: | It is recommended to add deionized water to prepare a working stock solution of approximately 0.5mg/ml and let the lyophilized pellet dissolve completely |
| Stability: | Store lyophilized protein at -20°C. Aliquot the product after reconstitution to avoid repeated freezing/thawing cycles. Reconstituted protein can be stored at 4°C for a limited period of time; it does not show any change after two weeks at 4°C. |
| Purity: | Greater than 95.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | ASSSPECACG RSHFTCAVSA LGECTCIPAQ WQCDGDNDCG DHSDEDGCIL PTCSPLDFHC DNGKCIRRSW VCDGDNDCED DSDEQDCPPR ECEEDEFPCQ NGYCIRSLWH CDGDNDCGDN SDEQCDMRKC SDKEFRCSDGS CIAEHWYCDG DTDCKDGSDE ENCPSAVPAP PCNLEEFQCA YGRCILDIYH CDGDDDCGDW SDESDCSSHQ PCRSGEFMCD SGLCINAGWRC DGDADCDDQS DERNCTTSMCT AEQFRCHSGR CVRLSWRCDG EDDCADNSDE ENCENTGSPQ CALDQFLCWN GRCIGQRKLC NGVNDCGDNS DESPQQNCRP RTGEENCNVN NGGCAQKCQM VRGAVQCTCH TGYRLTEDGH TCQDVNECAE EGYCSQGCTN SEGAFQCWCE TGYELRPDRR SCKALGPEPV LLFANRIDIR QVLPHRSEYT LLLNNLENAIA LDFHHRRELV FWSDVTLDRI LRANLNGSNV EEVVSTGLES PGGLAVDWVH DKLYWTDSGTSR IEVANLDGAHR KVLLWQNLEK PRAIALHPME GTIYWTDWGN TPRIEASSMD GSGRRIIADTHL FWPNGLTIDYAG RRMYWVDAKHHVI ERANLDGSHRK AVISQGLPHPFA ITVFEDSLYWTDW HTKSINSANKFTG KNQEIIRNKLHFPM DIHTLHPQRQPAGK NRCGDNNGGCTHLC LPSGQNYTCACPTG FRKISSHACAQ SLDKFLLFAR RMDIRRISFD TEDLSDDVIPL ADVRSAVALDW DSRDDHVYWT DVSTDTISRAKW DGTGQEVVVDT SLESPAGLAID WVTNKLYWTD AGTDRIEVAN TDGSMRTVLIW ENLDRPRDIVV EPMGGYMYWTDW GASPKIERAGM DASGRQVIISS NLTWPNGLAIDY GSQRLYWADAG MKTIEFAGLD GSKRKVLIGSQL PHPFGLTLYGE RIYWTDWQTKS IQSADRLTGLD RETLQENLEN LMDIHVFHRRR PPVSTPCAMEN GGCSHLCLRS PNPSGFSCTCP TGINLLSDGKT CSPGMNSFLI FARRIDIRMVSL DIPYFADVVVP INITMKNTIA VGVDPQEGKV YWSDSTLHRI SRANLDGSQH EDIITTGLQT TDGLAVDAIG RKVYWTDTGT NRIEVGNLDG SMRKVLVWQNL DSPRAIVLYH EMGFMYWTDWG ENAKLERSGM DGSDRAVLIN NNLGWPNGLT VDKASSQLLWA DAHTERIEAA DLNGANRHTL VSPVQHPYGLTL LDSYIYWTDW QTRSIHRADK GTGSNVILVR SNLPGLMDMQ AVDRAQPLGF NKCGSRNGGC SHLCLPRPSG FSCACPTGIQ LKGDGKTCDPS PETYLLFSSR GSIRRISLDT SDHTDVHVPV PELNNVISLDY DSVDGKVYYTD VFLDVIRRAD LNGSNMETVI GRGLKTTDGL AVDWVARNLYW TDTGRNTIEASR LDGSCRKVLINN SLDEPRAIAVF PRKGYLFWTDW GHIAKIERANLD GSERKVLINTDL GWPNGLTLDYDTR RIYWVDAHLDRI ESADLNGKLRQ VLVGHVSHPFAL TQQDRWIYWTD WQTKSIQRVD KYSGRNKETVL ANVEGLMDII VVSPQRQTGTN ACGVNNGGCT HLCFARASDFVC ACPDEPDSQPC SLVPGLVPPA PRATGMSEKS PVLPNTPPTT LYSSTTRTRT SLEEVEGRCS ERDARLGLCA RSNDAVPAAP GEGLHISKLH HHHHHHHHH |
LRP4 encodes a part of the low-density lipoprotein receptor-related protein family, which comprises of many evolutionarily conserved transmembrane proteins. LRP4 mediates SOST-dependent inhibition of bone formation. LRP4 takes part in the formation and the maintenance of the neuromuscular junction which is the synapse between skeletal muscle and motor neuron. LRP4 plays a role as a specific facilitator of SOST-mediated inhibition of Wnt signaling.
LRP4 Human Recombinant is a single, glycosylated polypeptide chain containing 1719 amino acids (21-1725a.a) and having a molecular mass of 191.6kDa (calculated). LRP4 is fused to a 14 a.a His tag (2 a.a on N-terminal and 12 a.a on C-terminal).
| UniProt Protein Function: | LRP4: Mediates SOST-dependent inhibition of bone formation. Functions as a specific facilitator of SOST-mediated inhibition of Wnt signaling. Plays a key role in the formation and the maintenance of the neuromuscular junction (NMJ), the synapse between motor neuron and skeletal muscle. Directly binds AGRIN and recruits it to the MUSK signaling complex. Mediates the AGRIN- induced phosphorylation of MUSK, the kinase of the complex. The activation of MUSK in myotubes induces the formation of NMJ by regulating different processes including the transcription of specific genes and the clustering of AChR in the postsynaptic membrane. Alternatively, may be involved in the negative regulation of the canonical Wnt signaling pathway, being able to antagonize the LRP6-mediated activation of this pathway. More generally, has been proposed to function as a cell surface endocytic receptor binding and internalizing extracellular ligands for degradation by lysosomes. Defects in LRP4 are the cause of Cenani-Lenz syndactyly syndrome (CLSS). It is a congenital malformation syndrome defined as complete and complex syndactyly of the hands combined with malformations of the forearm bones and similar manifestations in the lower limbs. Defects in LRP4 are the cause of sclerosteosis type 2 (SOST2). A sclerosing bone dysplasia characterized by a generalized hyperostosis and sclerosis leading to a markedly thickened skull, with mandible, ribs, clavicles and all long bones also being affected. Due to narrowing of the foramina of the cranial nerves, facial nerve palsy, hearing loss and atrophy of the optic nerves can occur. Sclerosteosis is clinically and radiologically very similar to van Buchem disease, mainly differentiated by hand malformations and a large stature in sclerosteosis patients. Belongs to the LDLR family. |
| UniProt Protein Details: | Protein type:Receptor, misc.; Membrane protein, integral; Cell surface Chromosomal Location of Human Ortholog: 11p11.2 Cellular Component: cell surface; cell soma; dendrite; postsynaptic density; integral to membrane; flotillin complex; neuromuscular junction Molecular Function:protein binding; protein homodimerization activity; apolipoprotein binding; calcium ion binding; receptor tyrosine kinase binding Biological Process: limb development; extracellular matrix organization and biogenesis; regulation of protein amino acid phosphorylation; Wnt receptor signaling pathway; dendrite morphogenesis; endocytosis; odontogenesis of dentine-containing teeth; dorsal/ventral pattern formation; synaptic growth at neuromuscular junction; hair follicle development; negative regulation of ossification; synapse organization and biogenesis; protein heterotetramerization; negative regulation of axonogenesis; embryonic digit morphogenesis; kidney development; proximal/distal pattern formation Disease: Myasthenic Syndrome, Congenital, 17; Cenani-lenz Syndactyly Syndrome; Sclerosteosis 2 |
| NCBI Summary: | This gene encodes a member of the low-density lipoprotein receptor-related protein family. The encoded protein may be a regulator of Wnt signaling. Mutations in this gene are associated with Cenani-Lenz syndrome. [provided by RefSeq, May 2010] |
| UniProt Code: | O75096 |
| NCBI GenInfo Identifier: | 157384998 |
| NCBI Gene ID: | 4038 |
| NCBI Accession: | NP_002325.2 |
| UniProt Secondary Accession: | O75096,Q4AC85, Q5KTZ5, B2RN39, |
| UniProt Related Accession: | O75096 |
| Molecular Weight: | 212,045 Da |
| NCBI Full Name: | low-density lipoprotein receptor-related protein 4 |
| NCBI Synonym Full Names: | low density lipoprotein receptor-related protein 4 |
| NCBI Official Symbol: | LRP4�� |
| NCBI Official Synonym Symbols: | CLSS; LRP-4; LRP10; MEGF7; SOST2�� |
| NCBI Protein Information: | low-density lipoprotein receptor-related protein 4; multiple epidermal growth factor-like domains 7 |
| UniProt Protein Name: | Low-density lipoprotein receptor-related protein 4 |
| UniProt Synonym Protein Names: | Multiple epidermal growth factor-like domains 7 |
| Protein Family: | Low-density lipoprotein receptor-related protein |
| UniProt Gene Name: | LRP4�� |
| UniProt Entry Name: | LRP4_HUMAN |
