LRP4 Antibody

說明書

中文名稱：

LRP4兔多克隆抗體
貨號：

CSB-PA013099ESR1HU
規格：

￥440
圖片：
Western blot
All lanes: LRP4 antibody at 1.39µg/ml + PC-3 whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 213 kDa
Observed band size: 213 kDa

Immunohistochemistry of paraffin-embedded human adrenal gland tissue using CSB-PA013099ESR1HU at dilution of 1:100
其他：

產品詳情
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靶點詳情

產品詳情

產品描述：

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.
產品名稱：

Rabbit anti-Homo sapiens (Human) LRP4 Polyclonal antibody
Uniprot No.：

O75096
基因名：

LRP4
別名：

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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Low-density lipoprotein receptor-related protein 4 protein (1500-1725AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC

推薦稀釋比：

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

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

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

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.
用途：

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

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
1. 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
2. LRP4 c.2552C>G (p.(T851R) variant was identified in the family with Chiari malformation type 1. PMID: 28513615
3. A novel splice variant in LRP4 (c.316+1G > A) segregated with Cenani-Lenz syndactyly phenotype in a five generations family. PMID: 28559208
4. LRP4 mutations alter Wnt/beta catenin signaling and cause limb and kidney malformations in Cenani-Lenz syndrome. PMID: 20381006
5. the first evidence suggesting that LRP4 is responsible for the retention of sclerostin in the bone environment in humans. PMID: 26751728
6. 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
7. 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
8. LRP4 is essential for maintaining the structural and functional activity of the neuromuscular junction. PMID: 25319686
9. 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
10. [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
11. 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
12. Cenani-Lenz syndrome in a large Pakistani pedigree is associated with a novel LRP4 missense mutation. PMID: 23664847
13. Data conclude that common variation in the LRP4 gene determines hip and whole body BMD PMID: 23321396
14. The roles of LRP4 in muscle fibers and motoneurons in neuromuscular junction formation have been dissected by cell-specific mutation. PMID: 22794264
15. 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
16. Lrp4 is a cis-acting ligand for MuSK PMID: 21969364
17. 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
18. 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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相關疾病：

Cenani-Lenz syndactyly syndrome (CLSS); Sclerosteosis 2 (SOST2); Myasthenic syndrome, congenital, 17 (CMS17)
亞細胞定位：

Cell membrane; Single-pass type I membrane protein.
蛋白家族：

LDLR family
組織特異性：

Expressed in bone; present in osteoblasts and osteocytes. No expression is observed in osteoclast. Expressed in several regions of the brain.
數據庫鏈接：

HGNC: 6696

OMIM: 212780

KEGG: hsa:4038

STRING: 9606.ENSP00000367888

UniGene: Hs.4930

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