Mouse LRP4 Monoclonal Antibody | anti-LRP4 antibody
LRP4 (cytoplasmic) Monoclonal Antibody
NCBI and Uniprot Product Information
NCBI Description
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 Description
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; 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
Research Articles on LRP4
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Product Notes
The LRP4 lrp4 (Catalog #AAA190923) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. AAA Biotech's LRP4 can be used in a range of immunoassay formats including, but not limited to, Immunoblot (IB). Immunoblotting: Use at 1ug/ml. Degradation products of approx. 100kDa are also detected. Researchers should empirically determine the suitability of the LRP4 lrp4 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "LRP4, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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