Gene Symbol: Dst
Description: dystonin
Alias: dystonin, bullous pemphigoid antigen 1
Species: rat
Products:     Dst

Top Publications

  1. Guo L, Degenstein L, Dowling J, Yu Q, Wollmann R, Perman B, et al. Gene targeting of BPAG1: abnormalities in mechanical strength and cell migration in stratified epithelia and neurologic degeneration. Cell. 1995;81:233-43 pubmed
    ..Unexpectedly, the mice also develop severe dystonia and sensory nerve degeneration typical of dystonia musculorum (dt/dt) mice. We show that in at least one other strain of dt/dt mice, BPAG1 gene is defective. ..
  2. Yang Y, Bauer C, Strasser G, Wollman R, Julien J, Fuchs E. Integrators of the cytoskeleton that stabilize microtubules. Cell. 1999;98:229-38 pubmed
    ..Its isoforms interconnect all three cytoskeletal networks, a feature apparently central to neuronal survival. ..
  3. Okumura M, Yamakawa H, Ohara O, Owaribe K. Novel alternative splicings of BPAG1 (bullous pemphigoid antigen 1) including the domain structure closely related to MACF (microtubule actin cross-linking factor). J Biol Chem. 2002;277:6682-7 pubmed
    BPAG1 (bullous pemphigoid antigen 1) was originally identified as a 230-kDa hemidesmosomal protein and belongs to the plakin family, because it consists of a plakin domain, a coiled-coil rod domain and a COOH-terminal intermediate ..
  4. Steiner Champliaud M, Schneider Y, Favre B, Paulhe F, Praetzel Wunder S, Faulkner G, et al. BPAG1 isoform-b: complex distribution pattern in striated and heart muscle and association with plectin and alpha-actinin. Exp Cell Res. 2010;316:297-313 pubmed publisher
    BPAG1-b is the major muscle-specific isoform encoded by the dystonin gene, which expresses various protein isoforms belonging to the plakin protein family with complex, tissue-specific expression profiles...
  5. Young K, Pool M, Kothary R. Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus. J Cell Sci. 2003;116:4543-55 pubmed
    Plakins are a family of giant cytoskeleton binding proteins. One member of this group is bullous pemphigoid antigen 1 (Bpag1)/dystonin, which has neuronal and muscle isoforms that consist of actin-binding and microtubule-binding domains ..
  6. Young K, Pinheiro B, Kothary R. A Bpag1 isoform involved in cytoskeletal organization surrounding the nucleus. Exp Cell Res. 2006;312:121-34 pubmed
    Bpag1/dystonin proteins are giant cytoskeletal interacting proteins postulated to cross-link cytoskeletal filaments and thereby maintain cellular integrity...
  7. Young K, Kothary R. Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neurons. Exp Cell Res. 2008;314:2750-61 pubmed publisher
    b>Dystonin/Bpag1 proteins are cytoskeletal linkers whose loss of function in mice results in a hereditary sensory neuropathy with a progressive loss of limb coordination starting in the second week of life...
  8. Hamill K, Hopkinson S, DeBiase P, Jones J. BPAG1e maintains keratinocyte polarity through beta4 integrin-mediated modulation of Rac1 and cofilin activities. Mol Biol Cell. 2009;20:2954-62 pubmed publisher
    ..These data indicate that the BPAG1e is required for efficient regulation of keratinocyte polarity and migration by determining the activation of Rac1. ..
  9. Pasdeloup D, McElwee M, Beilstein F, Labetoulle M, Rixon F. Herpesvirus tegument protein pUL37 interacts with dystonin/BPAG1 to promote capsid transport on microtubules during egress. J Virol. 2013;87:2857-67 pubmed publisher
    ..Here, we identify the spectraplakin protein, dystonin/BPAG1, an important cytoskeleton cross-linker involved in microtubule-based transport, as a binding partner of ..

More Information


  1. Bronson R, Sweet H, Spencer C, Davisson M. Genetic and age related models of neurodegeneration in mice: dystrophic axons. J Neurogenet. 1992;8:71-83 pubmed
    ..The mutant models of neuroaxonal dystrophy may prove useful in studies of the pathophysiology of DA in general and of specific inherited diseases of man, such as infantile neuroaxonal dystrophy and Hallervordin-Spatz disease. ..
  2. Boyer J, Bhanot K, Kothary R, Boudreau Larivière C. Hearts of dystonia musculorum mice display normal morphological and histological features but show signs of cardiac stress. PLoS ONE. 2010;5:e9465 pubmed publisher
    b>Dystonin is a giant cytoskeletal protein belonging to the plakin protein family and is believed to crosslink the major filament systems in contractile cells...
  3. Liu J, Ding J, Wu C, Bhagavatula P, Cui B, Chu S, et al. Retrolinkin, a membrane protein, plays an important role in retrograde axonal transport. Proc Natl Acad Sci U S A. 2007;104:2223-8 pubmed
    ..Our work lays the foundation for understanding fundamental issues of axonal transport and provides insights into the molecular mechanisms underlying human neurodegenerative disorders. ..
  4. Koster J, Geerts D, Favre B, Borradori L, Sonnenberg A. Analysis of the interactions between BP180, BP230, plectin and the integrin alpha6beta4 important for hemidesmosome assembly. J Cell Sci. 2003;116:387-99 pubmed
  5. Dowling J, Yu Q, Fuchs E. Beta4 integrin is required for hemidesmosome formation, cell adhesion and cell survival. J Cell Biol. 1996;134:559-72 pubmed
  6. Liu J, Ding J, Kowal A, Nardine T, Allen E, Delcroix J, et al. BPAG1n4 is essential for retrograde axonal transport in sensory neurons. J Cell Biol. 2003;163:223-9 pubmed
    Disruption of the BPAG1 (bullous pemphigoid antigen 1) gene results in progressive deterioration in motor function and devastating sensory neurodegeneration in the null mice...
  7. Honnappa S, Gouveia S, Weisbrich A, Damberger F, Bhavesh N, Jawhari H, et al. An EB1-binding motif acts as a microtubule tip localization signal. Cell. 2009;138:366-76 pubmed publisher
    ..Our findings establish a general "microtubule tip localization signal" (MtLS) and delineate a unifying mechanism for this subcellular protein targeting process. ..
  8. Favre B, Fontao L, Koster J, Shafaatian R, Jaunin F, Saurat J, et al. 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. 2001;276:32427-36 pubmed
    The bullous pemphigoid antigen 1 (eBPAG1) is a constituent of hemidesmosomes (HDs), cell-substrate adhesion complexes in stratified epithelia...