The Intermediate Filament Protein Peripherin Is the Specific Interaction Partner of Mouse BPAG1-n (Dystonin) in Neurons

@article{Leung1999TheIF,
  title={The Intermediate Filament Protein Peripherin Is the Specific Interaction Partner of Mouse BPAG1-n (Dystonin) in Neurons },
  author={Conrad L. Leung and Dongming Sun and Ronald K. H. Liem},
  journal={The Journal of Cell Biology},
  year={1999},
  volume={144},
  pages={435 - 446}
}
The dystonia musculorum (dt) mouse suffers from severe degeneration of primary sensory neurons. The mutated gene product is named dystonin and is identical to the neuronal isoform of bullous pemphigoid antigen 1 (BPAG1-n). BPAG1-n contains an actin-binding domain at its NH2 terminus and a putative intermediate filament-binding domain at its COOH terminus. Because the degenerating sensory neurons of dt mice display abnormal accumulations of intermediate filaments in the axons, BPAG1-n has been… CONTINUE READING

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Dystonia musculorum

  • L. W. Duchen, D. S. Falconer, S. J. Strich.
  • A hereditary neuropathy of mice affecting mainly…
  • 1963
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