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货号： bs-7505R-HRP 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-7505R-HRP
- 英文名称
- Anti-Plakophilin 1/HRP
- 中文名称
- 辣根过氧化物酶标记的桥粒斑菲素蛋白1抗体
- 别 名
- B6P; Band 6 protein; Plakophilin 1 (ectodermal dysplasia/skin fragility syndrome); PKP1_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Guinea Pig,
- 产品应用
- WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 83kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 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
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 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.
- 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 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.