Figure 1.

Time course of the developing neuropathy with subsequent remission of clinical symptoms in Miz1ΔPOZ animals.

The expression of the Cre recombinase starts at developmental day 12 under the desert hedgehog promotor. On day 30 postpartum, when the postnatal development of the peripheral nervous system is completed, no structural lesions are observed in the sciatic nerve of Miz1ΔPOZ animals. Between day 90 and day 120, an acute neuropathy with clinical symptoms of motor disabilities is observed. Outfoldings (arrows) of the myelin sheet are morphological hallmarks at this stage. Miz1ΔPOZ animals older than 120 days show a remission of the clinical symptoms and there is no further acute episode, at least up to one year. In older animals outfoldings are rare and the morphological hallmarks are hypomyelinated (scale bar) or demyelinated nerve fibers. Scale bar: 3 μm.