Fig. 3
CMT2D mice exhibit axonal transport defect prior to disease onset. a Experimental design. b A schematic picture of the microfluidic chamber used to evaluate axonal transport of mouse DRGs. DRGs of postnatal day 12 mice (Gars+/+ and GarsP234KY/+ littermates) were plated in the cell body compartment. Axons grew across the microgrooves into the axon compartment after 3 days in culture. QD655-labeled NGF (QD-NGF) was added (0.2 nM final concentration) to the axon compartment for axonal transport live imaging. c Representative kymographs of QD-NGF transport in Gars+/+ and GarsP234KY/+ mice-derived DRG axons. d, e Instantaneous velocities and pause durations of QD-NGF transport in DRG axons from Gars+/+ and GarsP234KY/+ littermates. DRGs were dissected and pooled from three mice for each genotype. n represents the number of QD-NGF-bearing endosomes measured for movements. Statistical analysis was done with two-tailed unpaired Student’s t-test. Data are presented as means ± s.d.
