Recombinant Human Low-density lipoprotein receptor-related protein 4 (LRP4), partial

中文名稱：

人LRP4重組蛋白
貨號：

CSB-EP013099HU
規格：

￥1344
圖片：
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

Greater than 90% as determined by SDS-PAGE.
基因名：

LRP4
Uniprot No.：

O75096
別名：

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

Homo sapiens (Human)
蛋白長度：

Cytoplasmic Domain
來源：

E.coli
分子量：

21.9kDa
表達區域：

1747-1905aa
氨基酸序列

YRHKKSKFTDPGMGNLTYSNPSYRTSTQEVKIEAIPKPAMYNQLCYKKEGGPDHNYTKEKIKIVEGICLLSGDDAEWDDLKQLRSSRGGLLRDHVCMKTDTVSIQASSGSLDDTETEQLLQEEQSECSSVHTAATPERRGSLPDTGWKHERKLSSESQV
Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
蛋白標簽：

N-terminal 6xHis-tagged
產品提供形式：

Liquid or Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
緩沖液：

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet & COA：

Please contact us to get it.

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