Recombinant Human Desmoglein-1 (DSG1), partial

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中文名稱：

人DSG1重組蛋白
貨號：

CSB-MP861938HUc7
說明書：

MSDS Datasheet
規(guī)格：

￥1140
圖片：
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
其他：

產(chǎn)品詳情
產(chǎn)品評價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

純度：

Greater than 95% as determined by SDS-PAGE.
生物活性：

Not Test
基因名：

DSG1
Uniprot No.：

Q02413
別名：

Cadherin family member 4;Desmosomal glycoprotein 1;DG1;DGI;Pemphigus foliaceus antigen
種屬：

Homo sapiens (Human)
蛋白長度：

Partial
來源：

Mammalian cell
分子量：

59.5 kDa
表達(dá)區(qū)域：

24-548aa
氨基酸序列

EFRIQVRDYNTKNGTIKWHSIRRQKREWIKFAAACREGEDNSKRNPIAKIHSDCAANQQVTYRISGVGIDQPPYGIFVINQKTGEINITSIVDREVTPFFIIYCRALNSMGQDLERPLELRVRVLDINDNPPVFSMATFAGQIEENSNANTLVMILNATDADEPNNLNSKIAFKIIRQEPSDSPMFIINRNTGEIRTMNNFLDREQYGQYALAVRGSDRDGGADGMSAECECNIKILDVNDNIPYMEQSSYTIEIQENTLNSNLLEIRVIDLDEEFSANWMAVIFFISGNEGNWFEIEMNERTNVGILKVVKPLDYEAMQSLQLSIGVRNKAEFHHSIMSQYKLKASAISVTVLNVIEGPVFRPGSKTYVVTGNMGSNDKVGDFVATDLDTGRPSTTVRYVMGNNPADLLAVDSRTGKLTLKNKVTKEQYNMLGGKYQGTILSIDDNLQRTCTGTININIQSFGNDDRTNTEPNTKITTNTGRQESTSSTNYDTSTTSTDSSQVYSSEPGNGAKDLLSDNVHFGP
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(biāo)簽：

C-terminal 6xHis-tagged
產(chǎn)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(fù)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℃/-80℃. 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(zhì)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(xiàng)：

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

Please contact us to get it.

產(chǎn)品評價(jià)

靶點(diǎn)詳情

功能：

Component of intercellular desmosome junctions. Involved in the interaction of plaque proteins and intermediate filaments mediating cell-cell adhesion.
基因功能參考文獻(xiàn)：
1. Here the authors identify a potential mechanism by which desmosomes assist the de-neddylating COP9 signalosome (CSN) in attenuating EGFR through an association between the Cops3 subunit of the CSN and desmosomal components, Desmoglein1 (Dsg1) and Desmoplakin (Dp), to promote epidermal differentiation. PMID: 28891468
2. Compared with immunocompetent patients, kidney transplant recipients systemically treated with calcineurin inhibitors and mycophenolate mofetil exhibited significantly reduced epidermal expression of Dsg1, a regulator of keratinocyte differentiation and barrier function. PMID: 27302902
3. Study identified DSG1 to be involved in the progressive intestinal injury associated with the development of Crohn's Disease complications via its effect on intestinal cell adhesion. PMID: 28490445
4. The authors report one recurrent and five novel DSG1 mutations, causing varying patterns of PPK, highlighting the clinical heterogeneity arising from mutations in this gene. PMID: 27534273
5. Authors report a new family of severe dermatitis, multiple allergies and metabolic wasting syndrome of Asian decent and expand the spectrum of mutations in the DSG1 gene. PMID: 27154412
6. Case Report: striate palmoplantar keratoderma showing transgrediens in a patient with heterozygote nonsense mutations in DSG1 and SERPINB7. PMID: 27786350
7. Genetic deletion of sdrD in S. aureus NCTC8325-4 through allelic replacement resulted in decreased bacterial adherence to Dsg1- expressing HaCaT cells in vitro. PMID: 26924733
8. The results of this study describes the fourth family with a biallelic mutation in the DSG1 gene associated with SAM syndrome, and emphasizes that the presence of PPK and skin fragility with or without additional symptoms should alert clinicians to consider DSG1 deficiency. PMID: 26288349
9. Desmoglein 1 mutation as cause of SAM syndrome PMID: 26169895
10. Desmoglein-1 regulates esophageal epithelial barrier function and immune responses in eosinophilic esophagitis. PMID: 24220297
11. Ectopic expression of Dsg1 in keratinocyte monolayers rescued the UVB-induced differentiation defect PMID: 24594668
12. The data showed that serum autoantibodies of patients, previously identified as Dsg1 and Dsg3 positive, are able to recognize continuous linear epitope regions of both Dsg1 and Dsg3 proteins using pin-bound overlapping peptides in modified ELISAs. PMID: 23297065
13. The loss of Bcr or MAL reduced levels of Dsg1 mRNA in keratinocytes, and ectopic expression of Dsg1 rescued defects in differentiation seen upon loss of Bcr or MAL signaling. PMID: 23940119
14. DSG1 deficiency was associated with increased expression of a number of genes encoding allergy-related cytokines PMID: 23974871
15. IgG autoantibodies against Dsg1 are mostly raised against preDsg1 and/or C-terminal domains of Dsg1 in healthy Tunisians in the endemic area of pemphigus foliaceus. PMID: 23489520
16. DSG1 and Erbin cooperate to repress MAPK signaling and promote keratinocyte differentiation. PMID: 23524970
17. we show that the LJM11 salivary protein from the sand fly are recognized by pemphigus foliaceus autoantibodies against desmoglein 1 PMID: 22798673
18. Induced gene expression levels of plakoglobin, desmoglein-1 and desmoglein-2 correlated significantly with dilatation of intercellular spaces and basal cell hyperplasia in esophageal mucosa of patients with gastro-oesophageal reflux disease. PMID: 22276604
19. The identification of desmogleins 1 and 3, desmosomal adhesion glycoproteins, as targets in pemphigus, a fatal autoimmune blistering disease of the skin and mucous membranes, provided the first link between desmosomes, desmogleins, and human diseases. PMID: 22189787
20. anti-DSG1 response in fogo selvagem patients may be initiated by sensitization to an environmental allergen PMID: 21191415
21. KLK5 may promote metastatic dissemination of OSCC by promoting loss of junctional integrity through cleavage of desmoglein 1. PMID: 21163944
22. Data show that desmoglein 1 expression was required for the adhesive effects of EphA2. PMID: 20861311
23. Histone deacetylase inhibition up-regulates desmosomal cadherins and prevents the loss of adhesion induced by Dsg1 truncation. PMID: 21075858
24. localized on corneodesmosomes involved in adhesion PMID: 20116975
25. data demonstrate the role of epistasis between individual genes in Pemphigus foliaceus susceptibility and illustrate the genetic complexity of organ-specific autoimmune diseases; Epistasis between DSG1 and HLA class II genes PMID: 12058255
26. Staphylococcal exfoliative toxins act as serine proteases with extremely focused molecular specificity to cleave mouse and human desmoglein 1 (Dsg1) once after glutamic acid residue 381 between extracellular domains 3 and 4. PMID: 12093888
27. During high-level expression, keratin insertion at cell-cell contact sites was inhibited in desmoglein 1 but not in desmoglein 3, and desmoplakin was stained at cell-cell contact sites in desmoglein 3 but not in desmoglein 1. PMID: 12485422
28. Desmoglein 1 defined more differentiated cell populations, and was expressed in epidermal suprabasal cells, the inner root sheath, and the innermost layers of the outer root sheath. PMID: 12787134
29. Signaling pathway initiated by structural changes in the adherens junction in which adherens-junction-derived plakoglobin regulates nuclear transcription by antagonizing the binding of beta-catenin to T cell factor/lymphoid enhancer factor proteins. PMID: 12880414
30. Specificity of exfoliative toxin cleavage of desmoglein 1 resides not only in simple amino acid sequences but also in its calcium-dependent conformation. PMID: 12880431
31. a detailed epitope mapping reveals that the conformational epitopes recognized by IgG1 autoantibodies from these PF patients are restricted to the first 161 amino acids of Dsg1, whereas the linear epitopes are spread throughout the entire ectodomain. PMID: 14675185
32. Dsg1 promotes the formation of intercellular adhesion complexes. PMID: 15606501
33. a component of insect vector saliva, rather than the parasite itself may trigger an antibody response to EC-5 domain of desmoglein 1 PMID: 15610512
34. T-cell autoreactivity against Dsg1 is seen both in patients with pemphigus foliaceus and in healthy individuals PMID: 16026580
35. No significant relationship is revealed between genetic variants of autoantigen desmoglein 1 and pemphigus foliaceus in the Brazilian population. PMID: 16242304
36. desmoglein 1 is a novel caspase-3 target that regulates apoptosis in keratinocytes PMID: 16286477
37. perturbation of desmoglein 1 expression has a critical impact on the integrity of tissues experiencing strong mechanical stress. PMID: 16484817
38. An alternative transcript of DSG1, which contains a 101-bp insertion corresponding to the 3' end of DSG1-intron 6 and introducing a stop codon in the nucleotide sequence, is described. PMID: 17056584
39. Taken together, our results represent the first demonstration that anti-Dsg1 antibodies induce similar alterations on the subcellular distribution of Dsg1 irrespective of the disease where they come from. PMID: 17058228
40. novel splice site mutation in the DSG1 gene in palmoplantar keratoderma PMID: 17194569
41. Data show that both pemphigus foliaceus-IgG containing Dsg 1- but not Dsg 3-specific antibodies and pemphigus vulgaris-IgG with antibodies to Dsg 1 and Dsg 3 were effective in causing epidermal splitting in human skin and keratinocyte dissociation. PMID: 17640963
42. IgM anti-Dsg1 are common in fogo selvagem patients in their native environment and uncommon in other pemphigus phenotypes PMID: 17960181
43. findings show that mRNA of the DSG1 gene is present in normal thymus and that the expression of DSG1 transcript increases with age PMID: 18331528
44. The level of Dsg1, a marker of differentiating keratinocytes, was antagonistically regulated by two Ca-independent 'novel' nPKC isoforms; i.e. it increased by the differentiation-promoting nPKCdelta and decreased by the growth-promoting nPKCepsilon. PMID: 18637128
45. Decreased expression of desmoglein 1 is associated with a worse prognosis in head and neck squamous cell carcinoma patients PMID: 18752129
46. Direct sequencing of cDNA derived from affected skin in one patient failed to reveal a pathogenic mutation, suggesting that SPPK results from haploinsufficiency for DSG1. PMID: 19018793
47. The desmoglein-specific cytoplasmic region is an intrinsically disordered functional domain with an inducible structure that, along with the membrane proximal region, forms a flexible scaffold for cytoplasmic assembly at the desmosome. PMID: 19136012
48. Mutations in the desmoglein 1 gene have been indentified in five Pakistani families with striate palmoplantar keratoderma. PMID: 19157795
49. Pemphigus foliaceus is an autoimmune blistering skin disease characterized by the production of pathogenic IgG autoantibodies directed against desmoglein 1. PMID: 19453789
50. Data show that Dsg1 is required for maintaining epidermal tissue integrity in superficial layers, supports keratinocyte differentiation and suprabasal morphogenesis, and is required for suppression of epidermal growth factor receptor signaling. PMID: 19546243
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相關(guān)疾病：

Palmoplantar keratoderma 1, striate, focal, or diffuse (PPKS1); Erythroderma, congenital, with palmoplantar keratoderma, hypotrichosis, and hyper IgE (EPKHE)
亞細(xì)胞定位：

Cell membrane; Single-pass type I membrane protein. Cell junction, desmosome.
組織特異性：

Epidermis, tongue, tonsil and esophagus.
數(shù)據(jù)庫鏈接：

HGNC: 3048

OMIM: 125670

KEGG: hsa:1828

STRING: 9606.ENSP00000257192

UniGene: Hs.2633