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Dystonin (DST)

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BP240; BPA; BPAG1; CATX-15; DMH; MACF2; Dystonia musculorum protein; Bullous Pemphigoid Antigen 1; Hemidesmosomal plaque protein; 230 kDa bullous pemphigoid antigen

Dystonin (DST)
CCC C447
HGNC 1090
MGI 104627
PubMed 9570777
RGD 1306566
UniProt O94833
Wiki Dystonin

Dystonin, is a member of the plakin protein family of adhesion junction plaque proteins. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene, but the full-length nature of some variants has not been defined.

It has been known that some isoforms are expressed in neural and muscle tissue, anchoring neural intermediate filaments to the actin cytoskeleton, and some isoforms are expressed in epithelial tissue, anchoring keratin-containing intermediate filaments to hemidesmosomes. Consistent with the expression, mice defective for this gene show skin blistering and neurodegeneration.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPC447Hu01 Recombinant Dystonin (DST) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAC447Hu01 Polyclonal Antibody to Dystonin (DST) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Dystonin (DST) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dystonin (DST) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Dystonin (DST) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Dystonin (DST) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Dystonin (DST) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Dystonin (DST) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dystonin (DST) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Dystonin (DST) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Dystonin (DST) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Dystonin (DST) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Dystonin (DST) CLIA Kit Customized Service Offer
n/a ELISA Kit for Dystonin (DST) ELISA Kit Customized Service Offer
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  7. "Integrators of the cytoskeleton that stabilize microtubules."Cell 98:229-238(1999) [PubMed] [Europe PMC] [Abstract]
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  13. "The hemidesmosomal plaque. I. Characterization of a major constituent protein as a differentiation marker for certain forms of epithelia."Differentiation 45:207-220(1990) [PubMed] [Europe PMC] [Abstract]
  14. "Identification of a second protein product of the gene encoding a human epidermal autoantigen."Biochem. J. 300:851-857(1994) [PubMed] [Europe PMC] [Abstract]
  15. "An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments."Cell 86:655-665(1996) [PubMed] [Europe PMC] [Abstract]
  16. "The N terminus of the transmembrane protein BP180 interacts with the N-terminal domain of BP230, thereby mediating keratin cytoskeleton anchorage to the cell surface at the site of the hemidesmosome."Mol. Biol. Cell 11:277-286(2000) [PubMed] [Europe PMC] [Abstract]
  17. "Structural analysis of the predicted coiled-coil rod domain of the cytoplasmic bullous pemphigoid antigen (BPAG1). Empirical localization of the N-terminal globular domain-rod boundary."J. Biol. Chem. 271:9716-9722(1996) [PubMed] [Europe PMC] [Abstract]
  18. "The hemidesmosomal protein bullous pemphigoid antigen 1 and the integrin beta 4 subunit bind to ERBIN. Molecular cloning of multiple alternative splice variants of ERBIN and analysis of their tissue expression."J. Biol. Chem. 276:32427-32436(2001) [PubMed] [Europe PMC] [Abstract]
  19. "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]
  20. "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]
  21. "A quantitative atlas of mitotic phosphorylation."Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]
  22. "BPAG1e maintains keratinocyte polarity through beta4 integrin-mediated modulation of Rac1 and cofilin activities."Mol. Biol. Cell 20:2954-2962(2009) [PubMed] [Europe PMC] [Abstract]
  23. "BPAG1 isoform-b: complex distribution pattern in striated and heart muscle and association with plectin and alpha-actinin."Exp. Cell Res. 316:297-313(2010) [PubMed] [Europe PMC] [Abstract]
  24. "A homozygous nonsense mutation within the dystonin gene coding for the coiled-coil domain of the epithelial isoform of BPAG1 underlies a new subtype of autosomal recessive epidermolysis bullosa simplex."J. Invest. Dermatol. 130:1551-1557(2010) [PubMed] [Europe PMC] [Abstract]
  25. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
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  27. "Hereditary sensory autonomic neuropathy caused by a mutation in dystonin."Ann. Neurol. 71:569-572(2012) [PubMed] [Europe PMC] [Abstract]
  28. "Autosomal recessive epidermolysis bullosa simplex due to loss of BPAG1-e expression."J. Invest. Dermatol. 132:742-744(2012) [PubMed] [Europe PMC] [Abstract]
  29. "Identification of IGPR-1 as a novel adhesion molecule involved in angiogenesis."Mol. Biol. Cell 23:1646-1656(2012) [PubMed] [Europe PMC] [Abstract]
  30. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
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