mortality/aging
• mice die within 24 hours of birth likely due to respiratory defects
|
nervous system
• at E11.5, Vsx2+ spinal cords contain 57% fewer neurons than in wild-type mice, and by P0 no Chx+ interneurons are detected in the spinal cord
• at E13.5, Vsx2+ interneurons are reduced 4-fold compared to in wild-type mice with only newly generated Vsx2+ interneurons remaining in medial locations
• at P0, ventral vGlut2+ (Slc17a6+) neurons are decreased 30% compared to in wild-type mice
|
• V2a class excitatory interneurons are ablated
|
• the variability in burst amplitude and cycle period are greater in spinal cords and exhibit increased variability over additional cycles compared to in wild-type mice indicating that locomotor rhythms are destabilized
• however, commissurally projecting inhibitory glycinergic interneuron signaling, chemically-induced intrinsic rhythmogenic activity of spinal cord interneurons, and ipsilateral flexor-extensor alternations are normal
• signaling of left-right coordination is disrupted in the spinal cord compared to in wild-type mice
• neurally evoked locomotor rhythm initiation by the brainstem or sensory afferents is inhibited unlike in wild-type mice
|