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Desmoplakin 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. Homodimer. Interacts with COL17A1 (via cytoplasmic region). Associates (via C-terminal) with KRT5-KRT14 (via rod region), KRT8-KRT18 and VIM intermediate filaments. Interacts with DSC2. Isoform DPI is apparently an obligate constituent of all desmosomes. Isoform DPII resides predominantly in tissues and cells of stratified origin. Belongs to the plakin or cytolinker family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Cell adhesion; Cytoskeletal; Motility/polarity/chemotaxis
Chromosomal Location of Human Ortholog: 6p24.3
Cellular Component: cornified envelope; extracellular matrix; intermediate filament; nucleus; plasma membrane
Molecular Function: protein binding; protein binding, bridging; protein kinase C binding; RNA binding; structural constituent of cytoskeleton; structural molecule activity
Biological Process: epidermis development; intermediate filament organization; keratinization; keratinocyte differentiation; neutrophil degranulation; peptide cross-linking
Disease: Arrhythmogenic Right Ventricular Dysplasia, Familial, 8; Cardiomyopathy, Dilated, With Woolly Hair And Keratoderma; Cardiomyopathy, Dilated, With Woolly Hair, Keratoderma, And Tooth Agenesis; Epidermolysis Bullosa, Lethal Acantholytic; Keratosis Palmoplantaris Striata Ii; Skin Fragility-woolly Hair Syndrome
Reference #:  P15924 (UniProtKB)
Alt. Names/Synonyms: 250/210 kDa paraneoplastic pemphigus antigen; Desmoplakin; desmoplakin I; desmoplakin II; DESP; DP; DPI; DPII; DSP
Gene Symbols: DSP
Molecular weight: 331,774 Da
Basal Isoelectric point: 6.44  Predict pI for various phosphorylation states
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