神经退行性病
-
Figure 2｜Decreased Kif5α signals in neurites of motor neurons of wobbler mice at the stationary phase.
Due to their long axons, motor neurons are extremely dependent on axonal transport. As they are specifically affected by ALS, they are a very important target for further investigation. Since dysregulation of Kif5α expression in clinical wobbler mice was dysregulated at p40, we investigated the possible intracellular expression differences caused by this dysregulation using immunofluorescence staining and confocal laser microscopy. We found significantly lower kinesin spots in wobbler motor neuronal neurites compared with wild-type neurites (Figure 2). The signal distribution of Kif5α in wild-type and wobbler neurites generally followed the same trend, with the neurites of wobbler mice uniformly showing fewer kinesin spots and accumulations (Figure 3). It becomes obvious that kinesin signals could be detected as accumulations of molecules along the motor neuronal neurite of wild-type mice. The number of signals in the neurites of wobbler motor neurons was generally lower and accumulations occurred in a smaller quantity and did not reach the growth cone. Since existing literature suggests that Kif5α is important for mitochondrial transport and distribution, we performed experiments to qualitatively investigate the distribution of mitochondria in motor neurons of both genotypes at p40 (Additional Figure 2). While mitochondria in wild-type motor neurons are distributed relatively homogeneously over all branches with accumulations along the neurites (Additional Figure 2A), first hints could be gained on an altered distribution of mitochondria in Wobbler cells (Additional Figure 2B). However, these results still need to be supported by further studies.