Desmoplakin (Phospho Ser165/166) rabbit pAb

  • Catalog No.:YP1314
  • Applications:WB
  • Reactivity:Human;Mouse;Rat
    • Target:
    • Desmoplakin
    • Fields:
    • >>Arrhythmogenic right ventricular cardiomyopathy
    • Gene Name:
    • DSP
    • Protein Name:
    • Desmoplakin (Ser165/166)
    • Human Gene Id:
    • 1832
    • Human Swiss Prot No:
    • P15924
    • Mouse Swiss Prot No:
    • E9Q557
    • Immunogen:
    • Synthesized phosho peptide around human Desmoplakin (Ser165 and 166)
    • Specificity:
    • This antibody detects endogenous levels of Human Mouse Rat Desmoplakin (phospho-Ser165 or 166)
    • Formulation:
    • Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
    • Source:
    • Polyclonal, Rabbit,IgG
    • Dilution:
    • WB 1:1000-2000
    • Purification:
    • The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
    • Concentration:
    • 1 mg/ml
    • Storage Stability:
    • -15°C to -25°C/1 year(Do not lower than -25°C)
    • Other Name:
    • Desmoplakin (DP) (250/210 kDa paraneoplastic pemphigus antigen)
    • Observed Band(KD):
    • 300kD
    • Background:
    • 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],
    • Function:
    • 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
    • Subcellular Location:
    • 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). .
    • Expression:
    • 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.