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Previous Article | Next Article
The Journal of Neuroscience, May 1, 1999, 19(9):3396-3403
Peripheral Myelin Protein 22 and Protein Zero: a Novel Association in Peripheral Nervous System Myelin
Donatella D'Urso, Peter Ehrhardt, and Hans Werner Müller Molecular Neurobiology Laboratory, Department of Neurology, Heinrich-Heine-University, 40225 Düsseldorf, Germany
Mutations found in the two major glycosylated transmembrane proteins of the PNS myelin, the peripheral myelin protein zero (P0) and peripheral myelin protein 22 (PMP22), have been independently associated with the most common hereditary demyelinating peripheral neuropathies. Genotype-phenotype correlations in humans and transgenic animals have provided functional evidence that P0 and PMP22 are involved in formation and maintenance of compact myelin. Here, we demonstrate for the first time that P0 and PMP22 proteins form complexes in the myelin membrane, as shown by coimmunoprecipitation experiments, and that glycosylation is not involved in mediating these interactions. Complex formation was also detected when the two proteins were coexpressed in heterologous cells. In transfected cells, P0 and PMP22 are recruited and colocalize at the apposed plasma membranes of expressors as shown by confocal microscopy. These findings provide a new basis for a better understanding of myelin assembly and of the pathomechanisms involved in demyelinating peripheral neuropathies. Furthermore, these results propose a possible explanation why alterations in either of these molecules are sufficient to destabilize the myelin structure and cause a similar disease phenotype.
Key words: peripheral myelin protein 22; protein zero; protein complexes; immunoprecipitation; transfection; demyelinating peripheral neuropathies
Copyright © 1999 Society for Neuroscience 0270-6474/99/1993396-08$05.00/0
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