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Protein DSP PDB 1lm5.png
PDB rendering based on 1lm5.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols DSP ; DP; DPI; DPII
External IDs OMIM125647 MGI109611 HomoloGene37922 GeneCards: DSP Gene
RNA expression pattern
PBB GE DSP 200606 at tn.png
More reference expression data
Species Human Mouse
Entrez 1832 109620
Ensembl ENSG00000096696 ENSMUSG00000054889
UniProt P15924 E9Q557
RefSeq (mRNA) NM_001008844 NM_023842
RefSeq (protein) NP_001008844 NP_076331
Location (UCSC) Chr 6:
7.54 – 7.59 Mb
Chr 13:
38.15 – 38.2 Mb
PubMed search [1] [2]
Cell adhesion in desmosomes

Desmoplakin is a protein associated with desmosomes.[1]

Desmoplakin is a protein in humans that is encoded by the DSP gene.[2][3]


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. This is further sub divided into a region called the "Plakin domain" made up of repetitive domains called Spectrin repeats. A crystal structure of part of the plakin domain has been resolved,[4] while the entire plakin domain has been elucidated using Small angle X-ray scattering which revealed a non-linear structure, an unexpected result considering spectrin repeats are observed in linear orientations.[5] The C-terminus of desmoplakin binds with intermediate filaments. These are further sub divided to three homologous Plakin repeat domains (see PDBs below). 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.[3]


Desmoplakin has been shown to interact with:

See also[edit]


  1. ^ Bornslaeger E, Corcoran C, Stappenbeck T, Green K (August 1996). "Breaking the connection: displacement of the desmosomal plaque protein desmoplakin from cell-cell interfaces disrupts anchorage of intermediate filament bundles and alters intercellular junction assembly". J. Cell Biol. 134 (4): 985–1001. doi:10.1083/jcb.134.4.985. PMC 2120955. PMID 8769422. 
  2. ^ Arnemann J, Spurr N, Wheeler G, Parker A, Buxton R (October 1991). "Chromosomal assignment of the human genes coding for the major proteins of the desmosome junction, desmoglein DGI (DSG), desmocollins DGII/III (DSC), desmoplakins DPI/II (DSP), and plakoglobin DPIII (JUP)". Genomics 10 (3): 640–5. doi:10.1016/0888-7543(91)90446-L. PMID 1889810. 
  3. ^ a b "Entrez Gene: DSP desmoplakin". 
  4. ^ Choi H, Weis W (2011). "Crystal structure of a rigid four-spectrin-repeat fragment of the human desmoplakin plakin domain". J. Mol. Biol. 409 (5): 800–12. doi:10.1016/j.jmb.2011.04.046. PMC 3107870. PMID 21536047. 
  5. ^ Al-Jassar C, Knowles T, Jeeves M, Kami K, Behr E, Bikker H et al. (2011). "The nonlinear structure of the desmoplakin plakin domain and the effects of cardiomyopathy-linked mutations". J. Mol. Biol. 411 (5): 1049–61. doi:10.1016/j.jmb.2011.06.047. PMID 21756917. 
  6. ^ a b c Meng J, Bornslaeger E, Green K, Steinert P, Ip W (August 1997). "Two-hybrid analysis reveals fundamental differences in direct interactions between desmoplakin and cell type-specific intermediate filaments". J. Biol. Chem. 272 (34): 21495–503. PMID 9261168. 
  7. ^ Hofmann I, Mertens C, Brettel M, Nimmrich V, Schnölzer M, Herrmann H (July 2000). "Interaction of plakophilins with desmoplakin and intermediate filament proteins: an in vitro analysis". J. Cell. Sci. 113 ( Pt 13): 2471–83. PMID 10852826. 
  8. ^ Chen X, Bonne S, Hatzfeld M, van Roy F, Green K (March 2002). "Protein binding and functional characterization of plakophilin 2. Evidence for its diverse roles in desmosomes and beta -catenin signaling". J. Biol. Chem. 277 (12): 10512–22. doi:10.1074/jbc.M108765200. PMID 11790773. 
  9. ^ Kowalczyk A, Navarro P, Dejana E, Bornslaeger E, Green K, Kopp D et al. (October 1998). "VE-cadherin and desmoplakin are assembled into dermal microvascular endothelial intercellular junctions: a pivotal role for plakoglobin in the recruitment of desmoplakin to intercellular junctions". J. Cell. Sci. 111 ( Pt 20): 3045–57. PMID 9739078. 
  10. ^ Kowalczyk A, Bornslaeger E, Borgwardt J, Palka H, Dhaliwal A, Corcoran C et al. (November 1997). "The amino-terminal domain of desmoplakin binds to plakoglobin and clusters desmosomal cadherin-plakoglobin complexes". J. Cell Biol. 139 (3): 773–84. PMC 2141713. PMID 9348293. 

Further reading[edit]

External links[edit]