Recombinant Human Dystroglycan (DAG1), partial

^{In
Stock}

貨號：

CSB-BP613486HU
說明書：

MSDS Datasheet
規格：

￥3168
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：

DAG1
Uniprot No.：

Q14118
別名：

Dystrophin-associated glycoprotein 1
種屬：

Homo sapiens (Human)
蛋白長度：

Partial
來源：

Baculovirus
分子量：

71.7 kDa
表達區域：

30-653aa
氨基酸序列

HWPSEPSEAVRDWENQLEASMHSVLSDLHEAVPTVVGIPDGTAVVGRSFRVTIPTDLIASSGDIIKVSAAGKEALPSWLHWDSQSHTLEGLPLDTDKGVHYISVSATRLGANGSHIPQTSSVFSIEVYPEDHSELQSVRTASPDPGEVVSSACAADEPVTVLTVILDADLTKMTPKQRIDLLHRMRSFSEVELHNMKLVPVVNNRLFDMSAFMAGPGNAKKVVENGALLSWKLGCSLNQNSVPDIHGVEAPAREGAMSAQLGYPVVGWHIANKKPPLPKRVRRQIHATPTPVTAIGPPTTAIQEPPSRIVPTPTSPAIAPPTETMAPPVRDPVPGKPTVTIRTRGAIIQTPTLGPIQPTRVSEAGTTVPGQIRPTMTIPGYVEPTAVATPPTTTTKKPRVSTPKPATPSTDSTTTTTRRPTKKPRTPRPVPRVTTKVSITRLETASPPTRIRTTTSGVPRGGEPNQRPELKNHIDRVDAWVGTYFEVKIPSDTFYDHEDTTTDKLKLTLKLREQQLVGEKSWVQFNSNSQLMYGLPDSSHVGKHEYFMHATDKGGLSAVDAFEIHVHRRPQGDRAPARFKAKFVGDPALVLNDIHKKIALVKKLAFAFGDRNCSTITLQNITRG
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-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：

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
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：

The dystroglycan complex is involved in a number of processes including laminin and basement membrane assembly, sarcolemmal stability, cell survival, peripheral nerve myelination, nodal structure, cell migration, and epithelial polarization.; Extracellular peripheral glycoprotein that acts as a receptor for extracellular matrix proteins containing laminin-G domains. Receptor for laminin-2 (LAMA2) and agrin in peripheral nerve Schwann cells. Also acts as a receptor for laminin LAMA5.; Transmembrane protein that plays important roles in connecting the extracellular matrix to the cytoskeleton. Acts as a cell adhesion receptor in both muscle and non-muscle tissues. Receptor for both DMD and UTRN and, through these interactions, scaffolds axin to the cytoskeleton. Also functions in cell adhesion-mediated signaling and implicated in cell polarity.; (Microbial infection) Acts as a receptor for lassa virus and lymphocytic choriomeningitis virus glycoprotein and class C new-world arenaviruses. Acts as a Schwann cell receptor for Mycobacterium leprae, the causative organism of leprosy, but only in the presence of the G-domain of LAMA2.
基因功能參考文獻：
1. Study identified beta-dystroglycan as a substrate of WWP1 and found that the muscular dystrophy-causing mutation of WWP1 renders the enzyme hyperactive by relieving autoinhibition. PMID: 29635000
2. ISPD and FKTN are essential for the incorporation of ribitol into alpha-dystroglycan. PMID: 27194101
3. N-terminal alpha Dystroglycan ELISA signals were significantly reduced in Duchenne muscular dystrophy serum relative to serum from otherwise normal controls. PMID: 27854211
4. TMEM5 is a UDP-xylosyl transferase that elaborates the O-mannose glycan structure on alpha-dystroglycan. The authors demonstrate in a zebrafish model as well as in a human patient that defects in TMEM5 result in muscular dystrophy in combination with abnormal brain development. PMID: 27130732
5. Our results strongly suggest that the balance and integrity between the dystroglycan alpha and beta subunits are indispensable and responsible for the cell differentiation and proliferation in acute leukemia cells. PMID: 28591567
6. interation of DG with laminin and dynamin is involved in the regulation of AQP4 internalization PMID: 27788222
7. The Muscular Dystrophy Gene TMEM5 Encodes a Ribitol beta1,4-Xylosyltransferase Required for the Functional Glycosylation of Dystroglycan. PMID: 27733679
8. Data show that CD93 antigen proved to be phosphorylated on tyrosine 628 and 644 following cell adhesion on laminin through dystroglycan. PMID: 26848865
9. alpha-DG-N removal as an important posttranslational control of endometrial receptivity and uterine fluid alpha-DG-N as a potential biomarker for receptivity in women. PMID: 26077903
10. Phosphorylation within the cysteine-rich region of dystrophin enhances its association with beta-dystroglycan and identifies a potential novel therapeutic target for skeletal muscle wasting. PMID: 25082828
11. Novel mutations in DAG1 are associated with asymptomatic hyperCKemia with hypoglycosylation of alpha-dystroglycan. PMID: 25503980
12. Reduction of alpha-dystroglycan expression is correlated with glioma. PMID: 25139094
13. Depletion of DAG resulted in altered morphology and reduced properties of differentiated HL-60 cells, including chemotaxis, respiratory burst, phagocytic activities and markers of differentiation, implicating DAG as a protein involved in differentiation. PMID: 24792180
14. A report of a homozygous novel DAG1 missense mutation c.2006G>T in the beta-subunit of dystroglycan in two Libyan siblings with with a novel muscle-eye-brain disease-like phenotype with multicystic leucodystrophy. PMID: 24052401
15. The study provides evidence for at least three separate pools of dystroglycan complexes within myofibers that differ in composition and are differentially affected by loss of dystrophin. PMID: 23951345
16. These data suggest that proteolysis, tyrosine phosphorylation and translocation of dystroglycan to the nucleus resulting in altered gene transcription could be important mechanisms in the progression of prostate cancer. PMID: 24077328
17. T192M point-mutation in dystroglycan leads to a weaker interactions with laminin-1, which leads to hypoglycosylation and which finally leads to the limb girdle disease. PMID: 24361964
18. GTDC2 generates CTD110.6 antibody-reactive N-acetylglucosamine epitopes on the O-mannosylated alpha-dystroglycan. PMID: 24041696
19. the Dystroglycan-mediated cortical microtubule anchoring, the disruption of which initiates gastrulation EMT. PMID: 23940118
20. Loss of alpha-dystroglycan and increased CD133 expression are frequent events in human colon cancer and evaluation of CD133 expression could help to identify high-risk colon cancer patients. PMID: 22964035
21. Loss of LARGE2 disrupts functional glycosylation of alpha-dystroglycan in prostate cancer PMID: 23223448
22. The loss of dystroglycan during tumorigenesis leads to an increased ability for tumor growth. PMID: 22996647
23. novel role for HNK-1ST as a tumor suppressor controlling the functional glycans on alpha-DG and the importance of sulfate transfer in the glycosylation of alpha-DG. PMID: 22801424
24. Dynamics of expression patterns of dystroglycan in human glioblastoma PMID: 22307776
25. The virus-induced perturbation of alpha6beta1 integrin signalling critically depended on high-affinity Lassa virus binding to dystroglycan and dystroglycan's cytoplasmic domain. PMID: 22405130
26. Data suggest that throughout pregnancy, changes occur in expression and distribution of DAG1 and dystroglycan subunits in placental tissues undergoing placentation. PMID: 22138543
27. a mechanism by which Large competes with galactosyltransferase to target GlcNAc terminals to induce the functional glycans on alpha-DG PMID: 21347376
28. Reduced expression and altered localization of dystroglycan is common in pancreatic cancer, potentially contributing to the aggressive behavior of this disease. PMID: 20338590
29. the ligand-binding activity of alpha-dystroglycan is conferred primarily by LARGE modification at Thr-317 and -319, within the highly conserved first 18 amino acids of the mucin-like domain PMID: 21987822
30. These observations suggest that secreted alpha-DG-N may be transported via CSF and have yet unidentified effects on the nervous system. PMID: 21741360
31. LARGE has a role in inducing alpha-dystroglycan hyperglycosylation in skeletal and cardiac muscle PMID: 21203384
32. Ameloblastoma cells proliferate and are differentiated by capturing perlecan differentially with alpha-dystroglycan and integrin beta1, respectively PMID: 21255062
33. Data show that loss of nuclear p27(kip1) is a frequent event in human RCCs and is a powerful predictor of poor outcome which, in combination with low DG expression, could help to identify high-risk patients with clear cell RCC. PMID: 20626751
34. Beta-Dystroglycan interaction with caveolin-1 in smooth muscle is required for receptor-mediated Ca2+ release. PMID: 20736308
35. Twenty-five glycopeptides were characterized from human alpha-dystroglycan, which provide insight to the complex in vivo O-glycosylation of alpha-dystroglycan. PMID: 20507882
36. Biological role of dystroglycan in Schwann cell function, especially myelination, and its implications in diseases is reviewed. PMID: 20625412
37. Beta-dystroglycan follows a conventional Importin alpha/beta-dependent nuclear import pathway PMID: 20512930
38. Loss of alpha-dystroglycan expression is a frequent event in human oral squamous cell carcinoma PMID: 20350218
39. analysis of the main cleavage site involved in enzymatic processing of beta-dystroglycan recombinant ectodomain by MMP-9 PMID: 19946898
40. study identified phosphorylated O-mannosyl glycan on mucin-like domain of alpha-DG, which was required for laminin binding; patients with muscular dystrophy have defects in a postphosphoryl modification of this phosphorylated O-linked mannose PMID: 20044576
41. Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies. PMID: 12140558
42. Glomeruli possess large amounts of a specifically composed complex; this complex may undergo changes in human glomerular disease; and flattening of foot processes is directly associated with dissociation of laminin-dystroglycan complexes. PMID: 12386278
43. hAG-2 and hAG-3, human homologues of genes involved in differentiation, are associated with oestrogen receptor-positive breast tumours and interact with metastasis gene C4.4a and dystroglycan. PMID: 12592373
44. Expression is frequently reduced in human breast and colon cancers and is associated with tumor progression. PMID: 12598319
45. in human coxsackievirus B myocarditis a focal disruption of the DAG can principally occur and may contribute to the pathogenesis of human enterovirus-induced dilated cardiomyopathy PMID: 12920582
46. mutation of certain residues prevents both ezrin binding and the induction of actin-rich surface protrusions PMID: 15175275
47. Cells that are defective in components of the O-mannosylation pathway showed strikingly reduced lymphocytic choriomeningitis virus infectbility. PMID: 16254364
48. alpha-DG glycosylation may differ between neurons and glial cells in congenital musculaar dystrophy brains. PMID: 16466646
49. DG may be involved in the progression of primary brain tumors PMID: 16575202
50. Fukutin seems to bind to both the hypoglycosylated and fully glycosylated form of alpha-dystroglycan, and seems bind to the core area rather than the sugar chain of alpha-dystroglycan PMID: 17005282
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相關疾病：

Muscular dystrophy-dystroglycanopathy limb-girdle C9 (MDDGC9); Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A9 (MDDGA9)
亞細胞定位：

[Alpha-dystroglycan]: Secreted, extracellular space.; [Beta-dystroglycan]: Cell membrane; Single-pass type I membrane protein. Cytoplasm, cytoskeleton. Nucleus, nucleoplasm. Cell membrane, sarcolemma. Cell junction, synapse, postsynaptic cell membrane.
組織特異性：

Expressed in a variety of fetal and adult tissues. In epidermal tissue, located to the basement membrane. Also expressed in keratinocytes and fibroblasts.
數據庫鏈接：

HGNC: 2666

OMIM: 128239

KEGG: hsa:1605

STRING: 9606.ENSP00000312435

UniGene: Hs.76111