Analysis Tools
behavior/neurological
|
• increase in back/front step length ratio
|
paraparesis
(
J:240526
)
|
• failure to splay hindlimbs when suspended from tail
|
cellular
|
• muscle wasting
|
|
• failure to form uniform layer in cerebellum
|
growth/size/body
|
• at age 4 weeks
• at age P14 and 8 weeks
|
mortality/aging
| N |
• viable and embryos born at normal Mendelian ratio
|
|
• 50% of mice have to be culled by 6 months due to early-onset neurodysfunction
|
muscle
|
• muscle wasting
|
nervous system
|
• failure to form uniform layer in cerebellum
|
|
• failure to form uniform layer in cerebellum
• no evidence of apoptosis in cerebellum
|
|
• abnormal branching
|
|
• at age 3 months
|
|
• reactive gliosis as indicated by increased glial and complement-microglial markers in cerebellum of 3-months old mice
|
|
• endplate innervation
• increased numbers of endplates with terminal swellings and/or sproutings
• reduction in untethered synaptic vessels (SVs) at active zone in levator auris longus muscle
• enlarged endosomal and vacuolar structures in levator auris longus muscle
|
|
• reduced amplitude in cerebellum
• no change in frequency and decay kinetics
|
|
• significant number of neuromuscular junctions failed to respond to nerve stimulation or gave intermittent response
|
|
• increased mean frequency in muscle fibers
• increased half-decay time in some muscle fibers
• significantly increased incidence of giant MEPPs (GMEPPs)
|
skeleton
