Low-density lipoprotein receptor-related protein 4, Recombinant Protein

Multiple epidermal growth factor-like domains 7

For Research Use Only. Not for use in diagnostic procedures.

E Coli or Yeast or Baculovirus or Mammalian Cell

Greater than 90% as determined by SDS-PAGE. (lot specific)

Liquid containing glycerol

This protein contains an N-terminal tag and may also contain a C-terminal tag. Tag types are determined by various factors including tag-protein stability, please inquire for tag information.

Sterile filter available upon request.

Low endotoxin available upon request.

Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of LRP4 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Cardiovascular

(Note: Representative image, actual molecular weight may vary depending on Tag type and expression host)

22kD

LDL receptor related protein 4

low-density lipoprotein receptor-related protein 4

Low-density lipoprotein receptor-related protein 4

KIAA0816; LRP10; MEGF7; LRP-4  [Similar Products]

LRP4_HUMAN

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]

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.

Protein type: Membrane protein, integral; Cell surface; Receptor, misc.

Chromosomal Location of Human Ortholog: 11p11.2

Cellular Component: cell soma; cell surface; dendrite; flotillin complex; integral to membrane; neuromuscular junction; postsynaptic density

Molecular Function: apolipoprotein binding; calcium ion binding; protein binding; protein homodimerization activity; receptor tyrosine kinase binding

Biological Process: BMP signaling pathway; dendrite morphogenesis; dorsal/ventral pattern formation; embryonic digit morphogenesis; endocytosis; extracellular matrix organization and biogenesis; hair follicle development; kidney development; limb development; negative regulation of axonogenesis; negative regulation of ossification; odontogenesis of dentine-containing teeth; positive regulation of peptidyl-tyrosine phosphorylation; protein heterotetramerization; proximal/distal pattern formation; synapse organization and biogenesis; synaptic growth at neuromuscular junction; Wnt receptor signaling pathway

Disease: Cenani-lenz Syndactyly Syndrome; Myasthenic Syndrome, Congenital, 17; Sclerosteosis 2

Low density lipoprotein receptor-related protein 10.Ishikawa K., Fujimoto H., Kim D., Saeki S. Identification of high-molecular-weight proteins with multiple EGF-like motifs by motif-trap screening.Nakayama M., Nakajima D., Nagase T., Nomura N., Seki N., Ohara O.Genomics 51:27-34(1998) Human chromosome 11 DNA sequence and analysis including novel gene identification.Taylor T.D., Noguchi H., Totoki Y., Toyoda A., Kuroki Y., Dewar K., Lloyd C., Itoh T., Takeda T., Kim D.-W., She X., Barlow K.F., Bloom T., Bruford E., Chang J.L., Cuomo C.A., Eichler E., FitzGerald M.G., Jaffe D.B., LaButti K., Nicol R., Park H.-S., Seaman C., Sougnez C., Yang X., Zimmer A.R., Zody M.C., Birren B.W., Nusbaum C., Fujiyama A., Hattori M., Rogers J., Lander E.S., Sakaki Y.Nature 440:497-500(2006) LRP4 mutations alter Wnt/beta-catenin signaling and cause limb and kidney malformations in Cenani-Lenz syndrome.Li Y., Pawlik B., Elcioglu N., Aglan M., Kayserili H., Yigit G., Percin F., Goodman F., Nurnberg G., Cenani A., Urquhart J., Chung B.D., Ismail S., Amr K., Aslanger A.D., Becker C., Netzer C., Scambler P., Eyaid W., Hamamy H., Clayton-Smith J., Hennekam R., Nurnberg P., Herz J., Temtamy S.A., Wollnik B.Am. J. Hum. Genet. 86:696-706(2010) Bone overgrowth-associated mutations in the LRP4 gene impair sclerostin facilitator function.Leupin O., Piters E., Halleux C., Hu S., Kramer I., Morvan F., Bouwmeester T., Schirle M., Bueno-Lozano M., Fuentes F.J., Itin P.H., Boudin E., de Freitas F., Jennes K., Brannetti B., Charara N., Ebersbach H., Geisse S., Lu C.X., Bauer A., Van Hul W., Kneissel M.J. Biol. Chem. 286:19489-19500(2011)

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