The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
应用说明ICC/IF: Use at an assay dependent dilution.
IHC-Fr: Use at an assay dependent dilution.
WB: Use at an assay dependent dilution. Predicted molecular weight: 319 kDa.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
功能Major high molecular weight protein of desmosomes. Involved in the organization of the desmosomal cadherin-plakoglobin complexes into discrete plasma membrane domains and in the anchoring of intermediate filaments to the desmosomes.
组织特异性Isoform DPI is apparently an obligate constituent of all desmosomes. Isoform DPII resides predominantly in tissues and cells of stratified origin.
疾病相关Keratoderma, palmoplantar, striate 2 Cardiomyopathy, dilated, with woolly hair and keratoderma Arrhythmogenic right ventricular dysplasia, familial, 8 Skin fragility-woolly hair syndrome Epidermolysis bullosa, lethal acantholytic Cardiomyopathy, dilated, with woolly hair, keratoderma, and tooth agenesis
序列相似性Belongs to the plakin or cytolinker family. Contains 17 plectin repeats. Contains 1 SH3 domain. Contains 6 spectrin repeats.
结构域Its association with epidermal and simple keratins is dependent on the tertiary structure induced by heterodimerization of these intermediate filaments proteins and most likely involves recognition sites located in the rod domain of these keratins. The N-terminal region is required for localization to the desmosomal plaque and interacts with the N-terminal region of plakophilin 1. The three tandem plakin repeat regions in the C-terminus mediate binding to intermediate filaments.
翻译后修饰Ser-2849 is probably phosphorylated by a cAMP-dependent protein kinase. Phosphorylation on Ser-2849 probably affects its association with epidermal, simple cytokeratins and VIM intermediate filaments. Substrate of transglutaminase. Some glutamines and lysines are cross-linked to other desmoplakin molecules, to other proteins such as keratin, envoplakin, periplakin and involucrin, and to lipids like omega-hydroxyceramide (PubMed:9651377).
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).
Werner NS et al. Epidermolysis bullosa simplex-type mutations alter the dynamics of the keratin cytoskeleton and reveal a contribution of actin to the transport of keratin subunits. Mol Biol Cell15:990-1002 (2004).
Read more (PubMed: 14668478) »