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.
Organism species: Homo sapiens (Human)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | RPC203Hu01 | Recombinant Desmoplakin (DSP) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | PAC203Hu01 | Polyclonal Antibody to Desmoplakin (DSP) | WB; IHC; ICC; IP. |
MAC203Hu22 | Monoclonal Antibody to Desmoplakin (DSP) | WB; IHC; ICC; IP. | |
MAC203Hu21 | Monoclonal Antibody to Desmoplakin (DSP) | WB; IHC; ICC; IP. | |
Assay Kits | SEC203Hu | ELISA Kit for Desmoplakin (DSP) | Enzyme-linked immunosorbent assay for Antigen Detection. |
Organism species: Mus musculus (Mouse)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | n/a | Recombinant Desmoplakin (DSP) | Recombinant Protein Customized Service Offer |
Antibodies | n/a | Monoclonal Antibody to Desmoplakin (DSP) | Monoclonal Antibody Customized Service Offer |
n/a | Polyclonal Antibody to Desmoplakin (DSP) | Polyclonal Antibody Customized Service Offer | |
Assay Kits | n/a | CLIA Kit for Desmoplakin (DSP) | CLIA Kit Customized Service Offer |
n/a | ELISA Kit for Desmoplakin (DSP) | ELISA Kit Customized Service Offer |
Organism species: Rattus norvegicus (Rat)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | n/a | Recombinant Desmoplakin (DSP) | Recombinant Protein Customized Service Offer |
Antibodies | n/a | Monoclonal Antibody to Desmoplakin (DSP) | Monoclonal Antibody Customized Service Offer |
n/a | Polyclonal Antibody to Desmoplakin (DSP) | Polyclonal Antibody Customized Service Offer | |
Assay Kits | n/a | CLIA Kit for Desmoplakin (DSP) | CLIA Kit Customized Service Offer |
n/a | ELISA Kit for Desmoplakin (DSP) | ELISA Kit Customized Service Offer |
- "Molecular structure of the human desmoplakin I and II amino terminus."Proc. Natl. Acad. Sci. U.S.A. 89:544-548(1992) [PubMed] [Europe PMC] [Abstract]
- "Identification and characterization of DSPIa, a novel isoform of human desmoplakin."Cell Tissue Res. 341:121-129(2010) [PubMed] [Europe PMC] [Abstract]
- "The DNA sequence and analysis of human chromosome 6." Nature 425:805-811(2003) [PubMed] [Europe PMC] [Abstract]
- "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
- "Structure of the human desmoplakins. Implications for function in the desmosomal plaque."J. Biol. Chem. 265:2603-2612(1990) [PubMed] [Europe PMC] [Abstract]
- ErratumJ. Biol. Chem. 265:11406-11407(1990) [PubMed] [Europe PMC] [Abstract]
- "Striate palmoplantar keratoderma resulting from desmoplakin haploinsufficiency."J. Invest. Dermatol. 113:940-946(1999) [PubMed] [Europe PMC] [Abstract]
- "The amino-terminal domain of desmoplakin binds to plakoglobin and clusters desmosomal cadherin-plakoglobin complexes."J. Cell Biol. 139:773-784(1997) [PubMed] [Europe PMC] [Abstract]
- "Ceramides are bound to structural proteins of the human foreskin epidermal cornified cell envelope."J. Biol. Chem. 273:17763-17770(1998) [PubMed] [Europe PMC] [Abstract]
- "Haploinsufficiency of desmoplakin causes a striate subtype of palmoplantar keratoderma."Hum. Mol. Genet. 8:143-148(1999) [PubMed] [Europe PMC] [Abstract]
- ErratumHum. Mol. Genet. 8:943-943(1999)
- "Recessive mutation in desmoplakin disrupts desmoplakin-intermediate filament interactions and causes dilated cardiomyopathy, woolly hair and keratoderma."Hum. Mol. Genet. 9:2761-2766(2000) [PubMed] [Europe PMC] [Abstract]
- "Analysis of the interactions between BP180, BP230, plectin and the integrin alpha6beta4 important for hemidesmosome assembly."J. Cell Sci. 116:387-399(2003) [PubMed] [Europe PMC] [Abstract]
- "Interaction of the bullous pemphigoid antigen 1 (BP230) and desmoplakin with intermediate filaments is mediated by distinct sequences within their COOH terminus."Mol. Biol. Cell 14:1978-1992(2003) [PubMed] [Europe PMC] [Abstract]
- "Early death from cardiomyopathy in a family with autosomal dominant striate palmoplantar keratoderma and woolly hair associated with a novel insertion mutation in desmoplakin."J. Invest. Dermatol. 126:1651-1654(2006) [PubMed] [Europe PMC] [Abstract]
- "Combining protein-based IMAC, peptide-based IMAC, and MudPIT for efficient phosphoproteomic analysis."J. Proteome Res. 7:1346-1351(2008) [PubMed] [Europe PMC] [Abstract]
- "Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle."Mol. Cell 31:438-448(2008) [PubMed] [Europe PMC] [Abstract]
- "A quantitative atlas of mitotic phosphorylation."Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]
- "Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]
- "Large-scale proteomics analysis of the human kinome."Mol. Cell. Proteomics 8:1751-1764(2009) [PubMed] [Europe PMC] [Abstract]
- "Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions."Sci. Signal. 2:RA46-RA46(2009) [PubMed] [Europe PMC] [Abstract]
- "Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]
- "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
- "Mechanistic insights into arrhythmogenic right ventricular cardiomyopathy caused by desmocollin-2 mutations."Cardiovasc. Res. 90:77-87(2011) [PubMed] [Europe PMC] [Abstract]
- "A new hypo/oligodontia syndrome: Carvajal/Naxos syndrome secondary to desmoplakin-dominant mutations."J. Dent. Res. 90:58-64(2011) [PubMed] [Europe PMC] [Abstract]
- "System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation."Sci. Signal. 4:RS3-RS3(2011) [PubMed] [Europe PMC] [Abstract]
- "Molecular insights into arrhythmogenic right ventricular cardiomyopathy caused by plakophilin-2 missense mutations."Circ. Cardiovasc. Genet. 5:400-411(2012) [PubMed] [Europe PMC] [Abstract]
- "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
- "Structures of two intermediate filament-binding fragments of desmoplakin reveal a unique repeat motif structure."Nat. Struct. Biol. 9:612-620(2002) [PubMed] [Europe PMC] [Abstract]
- "Crystal structure of a rigid four-spectrin-repeat fragment of the human desmoplakin plakin domain."J. Mol. Biol. 409:800-812(2011) [PubMed] [Europe PMC] [Abstract]
- "Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy."Am. J. Hum. Genet. 71:1200-1206(2002) [PubMed] [Europe PMC] [Abstract]
- "Compound heterozygosity for non-sense and mis-sense mutations in desmoplakin underlies skin fragility/woolly hair syndrome."J. Invest. Dermatol. 118:232-238(2002) [PubMed] [Europe PMC] [Abstract]
- "A recessive mutation in desmoplakin causes arrhythmogenic right ventricular dysplasia, skin disorder, and woolly hair."J. Am. Coll. Cardiol. 42:319-327(2003) [PubMed] [Europe PMC] [Abstract]
- "Loss of desmoplakin tail causes lethal acantholytic epidermolysis bullosa."Am. J. Hum. Genet. 77:653-660(2005) [PubMed] [Europe PMC] [Abstract]
- "Clinical profile of four families with arrhythmogenic right ventricular cardiomyopathy caused by dominant desmoplakin mutations."Eur. Heart J. 26:1666-1675(2005) [PubMed] [Europe PMC] [Abstract]
- "Comprehensive desmosome mutation analysis in North Americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy."Circ. Cardiovasc. Genet. 2:428-435(2009) [PubMed] [Europe PMC] [Abstract]
- "Role of genetic testing in arrhythmogenic right ventricular cardiomyopathy/dysplasia."Clin. Genet. 77:37-48(2010) [PubMed] [Europe PMC] [Abstract]
- "Expanding the phenotype associated with a desmoplakin dominant mutation: Carvajal/Naxos syndrome associated with leukonychia and oligodontia."Int. J. Cardiol. 161:50-52(2012) [PubMed] [Europe PMC] [Abstract]