Recombinant Human Platelet glycoprotein Ib beta chain (GP1BB)

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中文名稱：

人GP1BB重組蛋白
貨號：

CSB-CF009686HU
說明書：

MSDS Datasheet
規格：

￥9720
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 85% as determined by SDS-PAGE.
基因名：

GP1BB
Uniprot No.：

P13224
別名：

GP1BB; Platelet glycoprotein Ib beta chain; GP-Ib beta; GPIb-beta; GPIbB; Antigen CD42b-beta; CD antigen CD42c
種屬：

Homo sapiens (Human)
蛋白長度：

Full Length of Mature Protein
來源：

in vitro E.coli expression system
分子量：

39.3 kDa
表達區域：

26-206aa
氨基酸序列

CPAPCSCAGTLVDCGRRGLTWASLPTAFPVDTTELVLTGNNLTALPPGLLDALPALRTAHLGANPWRCDCRLVPLRAWLAGRPERAPYRDLRCVAPPALRGRLLPYLAEDELRAACAPGPLCWGALAAQLALLGLGLLHALLLVLLLCRLRRLRARARARAAARLSLTDPLVAERAGTDES
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 10xHis-SUMO-tagged and C-terminal Myc-tagged
產品提供形式：

Liquid or Lyophilized powder Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 662
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：

Lyophilized from 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：

3-7 business days
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：

Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.
基因功能參考文獻：
1. Rare variants in GP1BB are responsible for autosomal dominant macrothrombocytopenia. PMID: 28064200
2. Data show that localization of the GP Ib-IX complex to the lipid domain is mediated by GP Ibbeta and GP IX transmembrane domains. PMID: 26203189
3. Low levels of CD9 coincidental with a novel nonsense mutation in glycoprotein Ibbeta in a patient with Bernard-Soulier syndrome. PMID: 26275786
4. a suspicion of 22q11.2 deletion is warranted in pediatric BSS patients with a mutation in the GPIbbeta gene, even without remarkable symptoms. PMID: 23566026
5. GPIbbeta missense mutations from Bernard-Soulier syndrome were examined for changes to GPIb-IX complex surface expression. Mutations A108P and P74R were found to maintain normal secretion/folding of GPIbbeta(E) but were unable to support GPIX surface expression PMID: 21908432
6. A novel variant of Bernard Soulier syndrome is described in which Ser23 of GPIbbeta is substituted by a Stop codon causing a premature termination of translation. PMID: 19484238
7. A 14-month-old boy with Bernard-Soulier syndrome was found to be homozygous for a nonsense mutation (c.423C > A) in the glycoprotein Ib-beta. PMID: 22343686
8. Identify TRAF4 as a novel binding partner for GPIb-IX-V and GPVI in human platelets. PMID: 20946164
9. putative convex surface of the LRR domain in GPIX is sufficient, in the context of full-length subunit, to mediate its association with GPIbbeta PMID: 19566547
10. Lateral clustering of platelet GP Ib-IX complexes leads to up-regulation of the adhesive function of integrin alpha IIbbeta 3 PMID: 11812775
11. Role of GPIbbeta in modulating vWF mediated platelet adhesion. PMID: 11816713
12. A GP1BB missense mutation suppresses GPIb/IX complex expression and is found to cause Bernard-Soulier Syndrome in the homozygous form and giant platelets in the heterozygous form. PMID: 11816714
13. The cysteine knot of platelet GPIb beta is critical for the interaction of GPIb beta with GPIX. PMID: 12036872
14. PKA-mediated phosphorylation of GPIbbeta at Ser(166) negatively regulates VWF binding to GPIb-IX and is one of the mechanisms by which PKA mediates platelet inhibition PMID: 12361948
15. The GPIb beta intracellular domain has a role in controlling the adhesive properties of the GPIb/V/IX complex through phosphorylation of GPIb beta Ser166 and point to the existence of cross-talk between the GPIb beta and GPIb alpha intracellular domains. PMID: 12522011
16. novel hemizygous variant of Bernard-Soulier Syndrome in which Pro29 in one GPIbbeta allele is substituted by a Leu PMID: 12529755
17. To determine the sequence in the beta3 cytoplasmic domain that is critical to integrin signaling, cell lines were established that coexpress the platelet receptor for GP1BB. PMID: 12860973
18. identified novel GPIbbeta mutation is responsible for the Bernard-Soulier syndrome phenotype and provide an explanation for the molecular mechanism underlying the reduced platelet content of GPIb-IX complex in the heterozygous individuals studied PMID: 12958615
19. The SEPT5 gene resides approximately 250 nucleotides 5' to the GP Ibbeta gene and has been associated with modulating exocytosis from neurons and platelets as part of a presynaptic protein complex PMID: 15213102
20. Velocardiofacial syndrome patients have in-vitro defects in platelet function that may increase their risk of bleeding during surgery. PMID: 18064328
21. the N1421K substitution in the VWF affects the GPIb binding site or a recognition element by a conformational change of the A1 domain. PMID: 18637125
22. proplatelet formation in human megakaryocytes undergoes a complex spatio-temporal regulation orchestrated by adhesive proteins, GPIb-IX-V and myosin IIA PMID: 18752571
23. novel Ser 23 Stop mutation in GPIbbeta is responsible of BSS in the studied family and hampers the complex to form on the platelets surface. PMID: 18825380
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相關疾病：

Bernard-Soulier syndrome (BSS)
亞細胞定位：

Membrane; Single-pass type I membrane protein.
組織特異性：

Expressed in heart and brain.
數據庫鏈接：

HGNC: 4440

OMIM: 138720

KEGG: hsa:2812

STRING: 9606.ENSP00000383382

UniGene: Hs.283743