Desmoplakin (Phospho Ser165/166) rabbit pAb
- 货号：YP1314
- 应用：WB
- 种属：Human;Mouse;Rat
- 简介：
- >>Arrhythmogenic right ventricular cardiomyopathy
- 蛋白名称：
- Desmoplakin (Ser165/166)
- 免疫原：
- Synthesized phosho peptide around human Desmoplakin (Ser165 and 166)
- 特异性：
- This antibody detects endogenous levels of Human Mouse Rat Desmoplakin (phospho-Ser165 or 166)
- 组成：
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 来源：
- Polyclonal, Rabbit,IgG
- 纯化工艺：
- The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
- 储存：
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称：
- Desmoplakin (DP) (250/210 kDa paraneoplastic pemphigus antigen)
- 背景：
- This gene encodes a protein that anchors intermediate filaments to desmosomal plaques and forms an obligate component of functional desmosomes. Mutations in this gene are the cause of several cardiomyopathies and keratodermas, including skin fragility-woolly hair syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016],
- 功能：
- disease:Defects in DSP are the cause of dilated cardiomyopathy with woolly hair and keratoderma (DCWHK) [MIM:605676]; also known as Carvajal syndrome or palmoplantar keratoderma with left ventricular cardiomyopathy and woolly hair. DCWHK is an autosomal recessive cardiocutaneous syndrome characterized by a generalized striate keratoderma particularly affecting the palmoplantar epidermis, woolly hair, and dilated left ventricular cardiomyopathy.,disease:Defects in DSP are the cause of epidermolysis bullosa lethal acantholytic (EBLA) [MIM:609638]. EBLA is characterized by severe fragility of skin and mucous membranes. The phenotype is lethal in the neonatal period because of immense transcutaneous fluid loss. Typical features include universal alopecia, neonatal teeth, and nail loss. Histopathology of the skin shows suprabasal clefting and acantholysis throughout the spinous layer, mimicki
- 细胞定位：
- Cell junction, desmosome . Cytoplasm, cytoskeleton . Cell membrane . Innermost portion of the desmosomal plaque. Colocalizes with epidermal KRT5-KRT14 and simple KRT8-KRT18 keratins and VIM intermediate filaments network (PubMed:12802069). Localizes at the intercalated disk in cardiomyocytes (By similarity). .
- 组织表达：
- Expressed in oral mucosa (at protein level) (PubMed:30479852). Expressed in arrector pili muscle (at protein level) (PubMed:29034528). ; [Isoform DPI]: Apparently an obligate constituent of all desmosomes.; [Isoform DPII]: Resides predominantly in tissues and cells of stratified origin.
