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Desmoplakin (DSP)

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DPI; DPII; KPPS2; PPKS2; DP; 250/210 kDa paraneoplastic pemphigus antigen

Desmoplakin (DSP)
CCC C203
HGNC 3052
MGI 109611
PubMed 12101406
RGD 1305794
UniProt P15924
Wiki Desmoplakin
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.
MAC203Hu23 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
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  11. ErratumHum. Mol. Genet. 8:943-943(1999)
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  18. "A quantitative atlas of mitotic phosphorylation."Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]
  19. "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]
  20. "Large-scale proteomics analysis of the human kinome."Mol. Cell. Proteomics 8:1751-1764(2009) [PubMed] [Europe PMC] [Abstract]
  21. "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]
  22. "Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]
  23. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  24. "Mechanistic insights into arrhythmogenic right ventricular cardiomyopathy caused by desmocollin-2 mutations."Cardiovasc. Res. 90:77-87(2011) [PubMed] [Europe PMC] [Abstract]
  25. "A new hypo/oligodontia syndrome: Carvajal/Naxos syndrome secondary to desmoplakin-dominant mutations."J. Dent. Res. 90:58-64(2011) [PubMed] [Europe PMC] [Abstract]
  26. "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]
  27. "Molecular insights into arrhythmogenic right ventricular cardiomyopathy caused by plakophilin-2 missense mutations."Circ. Cardiovasc. Genet. 5:400-411(2012) [PubMed] [Europe PMC] [Abstract]
  28. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  29. "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]
  30. "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]
  31. "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]
  32. "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]
  33. "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]
  34. "Loss of desmoplakin tail causes lethal acantholytic epidermolysis bullosa."Am. J. Hum. Genet. 77:653-660(2005) [PubMed] [Europe PMC] [Abstract]
  35. "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]
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  38. "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]