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© The Rockefeller University Press, 0021-9525/1999//435 $5.00
The Journal of Cell Biology, Volume 144, Number 3, , 1999 435-446

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



Conrad L. Leung*, Dongming Sun{ddagger}, and Ronald K.H. Liem{ddagger}

* Departments of Biochemistry and Molecular Biophysics and {ddagger} Departments of Pathology and Anatomy and Cell Biology, Columbia University College of Physicians and Surgeons, New York 10032

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 postulated to organize the neuronal cytoskeleton by interacting with both the neurofilament triplet proteins (NFTPs) and microfilaments. In this paper we show by a variety of methods that the COOH-terminal tail domain of mouse BPAG1 interacts specifically with peripherin, but in contrast to a previous study (Yang, Y., J. Dowling, Q.C. Yu, P. Kouklis, D.W. Cleveland, and E. Fuchs. 1996. Cell. 86:655–665), mouse BPAG1 fails to associate with full-length NFTPs. The tail domains interfered with the association of the NFTPs with BPAG1. In dt mice, peripherin is present in axonal swellings of degenerating sensory neurons in the dorsal root ganglia and is downregulated even in other neural regions, which have no obvious signs of pathology. Since peripherin and BPAG1-n also display similar expression patterns in the nervous system, we suggest that peripherin is the specific interaction partner of BPAG1-n in vivo.

Key Words: BPAG1 • dystonin • peripherin • neurofilament • intermediate filament

Abbreviations used in this paper: BPAG1, bullous pemphigoid antigen 1; CNS, central nervous system; DRG, dorsal root ganglia; dt, dystonia musculorum; GAD, GAL4 activation domain; GBD, GAL4 DNA-binding domain; IF, intermediate filament; NF, neurofilament; NFTPs, neurofilament triplet proteins; nIF, neuronal IF; PNS, peripheral nervous system.


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