Human Low-density lipoprotein receptor-related protein 4 ELISA Kit | LRP4 elisa kit
Human Low-density lipoprotein receptor-related protein 4 ELISA Kit
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: Cell surface; Membrane protein, integral; Receptor, misc.
Chromosomal Location of Human Ortholog: 11p11.2
Cellular Component: cell soma; cell surface; dendrite; flotillin complex; neuromuscular junction; plasma membrane; postsynaptic density; receptor complex
Molecular Function: protein binding; receptor tyrosine kinase binding; Wnt receptor activity; Wnt-protein binding
Biological Process: dendrite morphogenesis; kidney development; limb development; negative regulation of axonogenesis; negative regulation of ossification; protein heterotetramerization; synapse organization and biogenesis; synaptic growth at neuromuscular junction
Disease: Cenani-lenz Syndactyly Syndrome; Myasthenic Syndrome, Congenital, 17; Sclerosteosis 2
Research Articles on LRP4
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Product Notes
The Human LRP4 lrp4 (Catalog #AAA9426583) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9426583 ELISA Kit recognizes Human LRP4. It is sometimes possible for the material contained within the vial of "Low-density lipoprotein receptor-related protein 4, ELISA Kit" 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
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