PMID:10212299

Citation	D'Urso, D, Ehrhardt, P and Müller, HW (1999) Peripheral myelin protein 22 and protein zero: a novel association in peripheral nervous system myelin. J. Neurosci. 19:3396-403
Abstract	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.
Links	PubMed
Keywords	Animals; Base Sequence; Blotting, Western; Cell Membrane/metabolism; Cell Membrane/ultrastructure; DNA Primers; Electrophoresis, Polyacrylamide Gel; Glycosylation; HeLa Cells; Humans; Molecular Sequence Data; Myelin P0 Protein/genetics; Myelin P0 Protein/isolation & purification; Myelin P0 Protein/metabolism; Myelin Proteins/genetics; Myelin Proteins/isolation & purification; Myelin Proteins/metabolism; Myelin Sheath/chemistry; Protein Binding; Rats; Recombinant Proteins/isolation & purification; Recombinant Proteins/metabolism; Sciatic Nerve/chemistry; Transfection
edit table

Annotations

Gene product	Qualifier	GO Term	Evidence Code	with/from	Aspect	Extension	Notes	Status
RAT:MYP0		GO:0005886: plasma membrane	ECO:0000315:		C		Figure 4.	complete CACAO 9640
RAT:MYP0	part_of	GO:0005886: plasma membrane	ECO:0000315: mutant phenotype evidence used in manual assertion		C		Seeded From UniProt	complete

References

See Help:References for how to manage references in GONUTS.