桥粒斑菲素蛋白1抗体
规格：1mg/1ml
英文名： Plakophilin 1
别名： B6P; Band 6 protein; Plakophilin 1 (ectodermal dysplasia/skin fragility syndrome); PKP1_HUMAN.
分子量： 83kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Plakophi
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig,
细胞定位：细胞核 细胞膜
桥粒斑菲素蛋白1抗体产品介绍：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桥粒斑菲素蛋白1抗体 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桥粒斑菲素蛋白1抗体 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.
桥粒斑菲素蛋白1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：心血管 细胞生物 **学 神经生物学 信号转导 细胞粘附分子 细胞外基质
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid