Service Communautaire d'Information sur la Recherche et le Développement - CORDIS

Regulation of PMP22 expression

Transgenic mice have been generated to study the regulation of PMP22. This peripheral myelin protein is the culprit in the vast majority of motor and sensory neuropathies, which show an increased frequency in the elderly. In addition, some of the changes which peripheral nerves undergo during normal ageing are similar to those observed in peripheral neuropathies.

The 10 kilobases of 5'-flanking region of the PMP22 gene contains the necessary information to mirror its endogenous expression pattern in peripheral nerves during development and regeneration and in mouse models of demyelination due to genetic lesions. Transgene expression is strongly regulated during myelination, demyelination, and remyelination in Schwann cells, demonstrating the crucial influence of neuron-Schwann cell interactions in the regulation of PMP22.

During the last reporting period, regulatory regions of the PMP22 promoter that direct the temporal, spatial and cell-specific expression of the myelin protein have been identified. The mice also offer an unique model to investigate the regulation by neurosteroids of PMP22 expression, not only in cultured cells, but also in vivo.

Reported by

Swiss Federal Institute of Technology in Zurich
ETH Hoenggerberg
8093 Zürich
Switzerland