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Rabbit Anti-Plakophilin 1/Cy3 Conjugated antibody (bs-7505R-Cy3)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-7505R-Cy3
英文名稱 Rabbit Anti-Plakophilin 1/Cy3 Conjugated antibody
中文名稱 Cy3標記的橋粒斑菲素蛋白1抗體
別    名 B6P; Band 6 protein; Plakophilin 1 (ectodermal dysplasia/skin fragility syndrome); PKP1_HUMAN.   
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  細胞生物  免疫學  神經生物學  信號轉導  細胞粘附分子  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 83kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Plakophilin 1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
Plays a role in formation of desmosomal plaques and is found in desmosomes of most simple and stratified epithelia. Not found in cell types that have non-epithelial desmosomes. Absent in fibroblasts and other connective tissue types, including sarcomas.

Function:
Seems to play a role in junctional plaques. Contributes to epidermal morphogenesis.

Subcellular Location:
Nucleus. Cell junction, desmosome. Note: Nuclear and associated with desmosomes.

Tissue Specificity:
Isoform 2 is widely expressed. Isoform 1 is expressed in stratified squamous, complex, glandular duct and bladder epithelia.

DISEASE:
Defects in PKP1 are the cause of ectodermal dysplasia-skin fragility syndrome (EDSFS) [MIM:604536]; also known as McGrath syndrome. Ectodermal dysplasia defines a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. EDSFS is characterized by features of both cutaneous fragility and congenital ectodermal dysplasia affecting abnormalities in other epithelia or tissues. Desmosomes in the skin are small and poorly formed with widening of keratinocyte intercellular spaces and perturbed desmosome/keratin intermediate filament interactions.

Similarity:
Belongs to the beta-catenin family.
Contains 9 ARM repeats.

Database links:

UniProtKB/Swiss-Prot: Q13835.2

Entrez Gene: 5317 Human

Entrez Gene: 18772 Mouse

Omim: 601975 Human

SwissProt: Q13835 Human

SwissProt: P97350 Mouse

Unigene: 497350 Human

Unigene: 4494 Mouse

 



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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