桥粒斑菲素蛋白1抗体

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英文名称  Anti-Plakophilin 1

中文名称  桥粒斑菲素蛋白1抗体

别    名  B6P; Band 6 protein; Plakophilin 1 (ectodermal dysplasia/skin fragility syndrome); PKP1_HUMAN.  

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit  

克隆类型  polyclonal

交叉反应  Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig

产品类型  一抗    

研究领域  心血管 细胞生物 免疫学 神经生物学 信号转导 细胞粘附分子 细胞外基质  

蛋白分子量  predicted molecular weight: 83kDa

性    状  Lyophilized or Liquid

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免 疫 原  KLH conjugated synthetic peptide derived from human Plakophilin 1

亚    型  IgG

纯化方法  affinity purified by Protein A

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500

（石蜡切片需做抗原修复）

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存条件  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.

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

产品介绍 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

桥粒斑菲素蛋白1抗体SwissProt: P97350 Mouse

Unigene: 497350 Human

Unigene: 4494 Mouse