[Roles of axonal transport affected by K141N mutant HSP22 in the pathogenesis of CMT2L].

OBJECTIVE

To compare the axonal transport of wild-type (WT) and K141N mutant HSP22 in transfected primary cultured cortical neurons.

METHODS

The plasmid (pCAGGS-HA-wtHSP22 or pCAGGS-HA-K141NHSP22) with WT or K141N mutant HSP22 gene and a GFP-expressing plasmid (pEGFP-N1) were co-transfected respectively into primary cultured cortical neurons. The axonal transport of WT and K141N mutant HSP22 was observed. And the distance traveled by WT and K141N mutant HSP22 was analyzed.

RESULTS

The WT HSP22 was transported within axons and uniformly present throughout the entire length of axons. K141N mutant HSP22 failed to be transported to the same extent and was present only in cell body and proximal portion of axons. Analysis of distance traveled revealed that WT HSP22 traveled significantly further than the K141N mutant HSP22.

CONCLUSION

The axonal transport of K141N mutant HSP22 may play an important role in the pathogenesis of CMT2L.