Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) LRP4 Polyclonal antibody

Uniprot No.

O75096

Target Names

LRP4

Alternative Names

KIAA0816 antibody; LDLR dan antibody; Low density lipoprotein receptor related protein 4 antibody; Low-density lipoprotein receptor-related protein 4 antibody; LRP-4 antibody; LRP10 antibody; Lrp4 antibody; LRP4_HUMAN antibody; MEGF7 antibody; Multiple epidermal growth factor like domains 7 antibody; Multiple epidermal growth factor-like domains 7 antibody

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human Low-density lipoprotein receptor-related protein 4 protein (1500-1725AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Polyclonal

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Form

Liquid

Tested Applications

ELISA, WB, IHC

Recommended Dilution

Application	Recommended Dilution
WB	1:1000-1:5000
IHC	1:20-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Description

The immunization of rabbits with the recombinant human LRP4 protein (1500-1725aa), collection of the rabbit's blood to isolate the anti-serum, and final purification from rabbit anti-serum by antigen affinity purification causes the creation of the anti-LRP4 antibody.

This LRP4 antibody is a polyclonal antibody and occurs as an unconjugated IgG. It is only reactive with human LRP4 protein, which specifically interacts with the agrin glycoprotein and plays a critical role in the neuromuscular junction (NMJ) formation and maintenance. LRP4 is also involved in the Wnt signaling pathway, bone development, and the regulation of lipid metabolism. And this LRP4 antibody has been validated for use in ELISA, WB, and IHC applications.

Related Products

Target Background

Function

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. May play an essential role in the process of digit differentiation.

Gene References into Functions

In vitro experiments demonstrated that LRP4 downregulation significantly inhibited the colony formation, proliferation, migration, and invasion of the three papillary thyroid cancer cell lines. PMID: 29885843
LRP4 c.2552C>G (p.(T851R) variant was identified in the family with Chiari malformation type 1. PMID: 28513615
A novel splice variant in LRP4 (c.316+1G > A) segregated with Cenani-Lenz syndactyly phenotype in a five generations family. PMID: 28559208
LRP4 mutations alter Wnt/beta catenin signaling and cause limb and kidney malformations in Cenani-Lenz syndrome. PMID: 20381006
the first evidence suggesting that LRP4 is responsible for the retention of sclerostin in the bone environment in humans. PMID: 26751728
study presents 2 sibling fetuses with a prenatal lethal presentation of mesomelic limb reductions, oligosyndactyly, genitourinary malformation and compound heterozygosity for 2 novel truncating mutations in LRP4 PMID: 24924585
MuSK myasthenia gravis IgG4 disrupts the interaction of LRP4 with MuSK but both IgG4 and IgG1-3 can disperse preformed agrin-independent AChR clusters PMID: 24244707
LRP4 is essential for maintaining the structural and functional activity of the neuromuscular junction. PMID: 25319686
LRP4 is a new CMS disease gene and the 3rd beta propeller domain of LRP4 mediates two signaling pathways in a position-specific manner. PMID: 24234652
[review] Autoantibodies against LRP4 differentially alter neuromuscular transmission, demonstrating how myasthenia gravis can be classified according to the profile of the antibodies; management of myasthenia gravis patients can be adapted accordingly. PMID: 24530233
pathogenic IgG4 antibodies to MuSK bind to a structural epitope in the first Ig-like domain of MuSK, prevent binding between MuSK and Lrp4, and inhibit Agrin-stimulated MuSK phosphorylation. PMID: 24297891
Cenani-Lenz syndrome in a large Pakistani pedigree is associated with a novel LRP4 missense mutation. PMID: 23664847
Data conclude that common variation in the LRP4 gene determines hip and whole body BMD PMID: 23321396
The roles of LRP4 in muscle fibers and motoneurons in neuromuscular junction formation have been dissected by cell-specific mutation. PMID: 22794264
Data suggest that LRP4 and interaction between LRP4 and genes in the Wnt and BMP signaling pathways modulate bone phenotypes including peak bone mass and fracture, the clinical endpoint of osteoporosis. PMID: 21645651
Lrp4 is a cis-acting ligand for MuSK PMID: 21969364
the interaction of sclerostin with LRP4 is required to mediate the inhibitory function of sclerostin on bone formation, thus identifying a novel role for LRP4 in bone. PMID: 21471202
The present studies suggest that LRP10 may play a significant role in the brain physiology other than lipoprotein metabolism. PMID: 20005200

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Involvement in disease

Cenani-Lenz syndactyly syndrome (CLSS); Sclerosteosis 2 (SOST2); Myasthenic syndrome, congenital, 17 (CMS17)

Subcellular Location

Cell membrane; Single-pass type I membrane protein.

Protein Families

LDLR family

Tissue Specificity

Expressed in bone; present in osteoblasts and osteocytes. No expression is observed in osteoclast. Expressed in several regions of the brain.

Database Links

HGNC: 6696

OMIM: 212780

KEGG: hsa:4038

STRING: 9606.ENSP00000367888

UniGene: Hs.4930

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