The molecular function of Bexl in the regeneration of demyelinated neurons in PM

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(Oral Presentation at the Antwerp Consortium July 2009)

The molecular function of Bexl in the regeneration of demyelinated neurons in

PMP22 mice mutants.

M.R. Khazaei, H. Halfter and P. Young

Department of Neurology, University Hospital of Munster, Munster, Germany

The loss of the myelin sheath has significant effects on the organization

ofaxons and results in axonal degeneration. Demyelinated neurons undergo

characteristic changes in gene expression. Most of these genes are prerequisites

for axonal regeneration and survival but

their function in the context of demeylination and regeneration is in most cases

not well understood. The identification of regeneration associated proteins will

have an impact on innovative therapeutic approaches.

In the expression profile analysis of MNs from Pmp22-1 and

Pmp22 mice, two mouse genetic models for demyelinating neuropathies, we have

found that the expression of Bexl, a p75 interacting protein, is upregulated.

The expression of Bexl was also increased in MNs from mice after nerve injury

but we could not find any change in

the expression of Bexl in MAG-1 - mice.

Here we show that Bexl is responsible for the regulation of Rho activity in a

p75-dependent signalling pathway. This raises the possibility that Bexl may have

an important, function in axonal regeneration following damage through

reorganization of cytoskeleton.