DSP Antibody – Goat Polyclonal

Desmosomes are intercellular junctions that tightly link adjacent cells. Desmoplakin is an obligate component of functional desmosomes that anchors intermediate filaments to desmosomal plaques. The N-terminus of desmoplakin is required for localization to the desmosome and interacts with the N-terminal region of plakophilin 1 and plakoglobin. The C-terminus of desmoplakin binds with intermediate filaments. In the mid-region of desmoplakin, a coiled-coiled rod domain is responsible for homodimerization. Mutations in this gene are the cause of several cardiomyopathies and keratodermas as well as the autoimmune disease paraneoplastic pemphigus. [provided by RefSeq, Jul 2008]

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.

Protein type: Cell adhesion; Motility/polarity/chemotaxis; Cytoskeletal

Chromosomal Location of Human Ortholog: 6p24

Cellular Component: desmosome; mitochondrion; basolateral plasma membrane; plasma membrane; cornified envelope; intermediate filament; fascia adherens; nucleus

Molecular Function: protein binding, bridging; protein binding; protein kinase C binding; structural constituent of cytoskeleton; cell adhesion molecule binding; structural molecule activity

Biological Process: keratinocyte differentiation; cell-cell adhesion; epidermis development; wound healing; apoptosis; intermediate filament organization; peptide cross-linking; cell structure disassembly during apoptosis

Disease: Cardiomyopathy, Dilated, With Woolly Hair And Keratoderma; Arrhythmogenic Right Ventricular Dysplasia, Familial, 8; Keratosis Palmoplantaris Striata Ii; Cardiomyopathy, Dilated, With Woolly Hair, Keratoderma, And Tooth Agenesis; Skin Fragility-woolly Hair Syndrome; Epidermolysis Bullosa, Lethal Acantholytic