Desmoplakin (UniProt: P15924; also known as DP, 250/210 kDa paraneoplastic pemphigus antigen) is encoded by the DSP gene (Gene ID:1832) in human. Desmoplakin is a major high molecular weight homodimeric protein that is 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, which are prominent adhesive junctions present between many epithelial cells and cardiomyocytes. Desmoplakin is the most abundant of the desmosomal proteins and plays a key role in tissue morphogenesis and maintaining the integrity of tissue. Desmoplakin is composed of an N-terminal plakin domain, a central alpha-helical coiled-coil rod domain, and a C-terminal intermediate filament-binding domain. The N-terminal domain is required for desmosome association and the C-terminal binds to desmin. Three isoforms for Desmoplakin have been reported that are produced by alternative splicing. Desmoplakin 2 and 3 lack most of the central rod domain. Desmoplakin 1 is the dominant isoform in the cardiac tissue. Desmoplakin is phosphorylated by protein kinase A on serine 2849 and this phosphorylation affects its association with epidermal, simple cytokeratins, and VIM intermediate filaments. Mutations in DSP gene have been linked to DCWHK (Cardiomyopathy, dilated, with woolly hair and keratoderma) that is characterized by a generalized striate keratoderma particularly affecting the palmoplantar epidermis, woolly hair, and dilated left ventricular cardiomyopathy. Mice deficient in Desmoplakin perish around the time of implantation (E6.5) and show fewer desmosomes.
Synonyms: DP, 250/210 kDa paraneoplastic pemphigus antigen
Application: Western Blotting Analysis: A representative lot detected Desmoplakin in Western Blotting applications (Sobolik-Delmaire, T., et. al. (2006). J Biol Chem. 281(25):16962-70; Hall, C., et. al. (2009). Cell Commun Adhes. 16(1-3):15-27; Roberts, B.J., et. al. (2011). Exp Cell Res. 317(20):2814-22).Immunocytochemistry Analysis: A representative lot detected Desmoplakin in Immunocytochemistry applications (Roberts, B.J., et. al. (2011). Exp Cell Res. 317(20):2814-22; Sobolik-Delmaire, T., et. al. (2006). J Biol Chem. 281(25):16962-70; Roberts, B.J., et. al. (2016). J Biol Chem. 291(48):24857-24865).
Other Notes: Concentration: Please refer to lot specific datasheet.