Low Density Lipoprotein Receptor Related Protein 4 (LRP4), Polyclonal Antibody

MEGF7; LRP10; Multiple epidermal growth factor-like domains 7

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

Polyclonal

Rabbit

200ug/ml (lot specific)

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of anti-LRP4 antibody 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.

Western Blot (WB), Immunohistochemistry (IHC)

212,045 Da

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]

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

Chromosomal Location of Human Ortholog: 11p11.2

Cellular Component: cell soma; cell surface; dendrite; flotillin complex; integral component of membrane; neuromuscular junction; plasma membrane; postsynaptic density; receptor complex

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

Biological Process: BMP signaling pathway; dendrite morphogenesis; embryonic digit morphogenesis; endocytosis; 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; synaptic growth at neuromuscular junction

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

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