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Desmocollin?3 Recombinant Rabbit mAb (bsm-61022R)  
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產(chǎn)品編號 bsm-61022R
英文名稱 Desmocollin?3 Recombinant Rabbit mAb
中文名稱 橋粒糖蛋白3重組兔單抗
別    名 Desmocollin-3; DSC3_HUMAN; Cadherin family member 3; Desmocollin-4; HT-CP; CDHF3; DSC4  
抗體來源 Rabbit
克隆類型 Recombinant
克 隆 號 15H3
交叉反應(yīng) Mouse,Rat (predicted: Human)
產(chǎn)品應(yīng)用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 100 kDa
檢測分子量
細胞定位 細胞膜 
性    狀 Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human Desmocollin 3 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 The protein encoded by this gene is a calcium-dependent glycoprotein that is a member of the desmocollin subfamily of the cadherin superfamily. These desmosomal family members, along with the desmogleins, are found primarily in epithelial cells where they constitute the adhesive proteins of the desmosome cell-cell junction and are required for cell adhesion and desmosome formation. The desmosomal family members are arranged in two clusters on chromosome 18, occupying less than 650 kb combined. Mutations in this gene are a cause of hypotrichosis and recurrent skin vesicles disorder. The protein can act as an autoantigen in pemphigus diseases, and it is also considered to be a biomarker for some cancers. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, Apr 2014]

Function:
Component of intercellular desmosome junctions. Involved in the interaction of plaque proteins and intermediate filaments mediating cell-cell adhesion. May contribute to epidermal cell positioning (stratification) by mediating differential adhesiveness between cells that express different isoforms.

Subcellular Location:
Cell membrane. Cell junction > desmosome.

Tissue Specificity:
Epidermis, buccal mucosa, esophagus and cervix.

DISEASE:
Defects in DSC3 are the cause of hypotrichosis and recurrent skin vesicles (HRSV) [MIM:613102]. A disorder characterized by hypotrichosis and the appearance of recurrent skin vesicle formation. Affected individuals show sparse and fragile hair on scalp, as well as absent eyebrows and eyelashes. Vesicles filled with thin, watery fluid are observed on the scalp and skin of most of the boby. Mucosal vesicles are absent.

SWISS:
Q14574

Gene ID:
1825

Database links:

Entrez Gene: 1825 Human

Entrez Gene: 13507 Mouse

Entrez Gene: 307563 Rat

Omim: 600271 Human

SwissProt: Q14574 Human

SwissProt: P55850 Mouse

Unigene: 41690 Human

Unigene: 89935 Mouse

Unigene: 99931 Rat



Involvement in disease;Defects in DSC3 are the cause of hypotrichosis and recurrent skin vesicles (HRSV). A disorder characterized by hypotrichosis and the appearance of recurrent skin vesicle formation. Affected individuals show sparse and fragile hair on scalp, as well as absent eyebrows and eyelashes. Vesicles filled with thin, watery fluid are observed on the scalp and skin of most of the boby. Mucosal vesicles are absent.
產(chǎn)品圖片
25 ug total protein per lane of various lysates (see on figure) probed with Desmocollin 3 monoclonal antibody, unconjugated (bsm-61022R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
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