Table I.

Summary of the molecular, cellular, and behavioral changes in TDP-43 Tg+/+ mice as compared to the WT

  2-mo Tg 6-mo Tg 
Cognitive functions 
 Water maze test decrease na 
 Fear conditioning task decrease na 
 Novel object recognition test decrease decrease 
Motor functions 
 Limb-clasping reflex similar to WT increase 
 Rotarod testing similar to WT decrease 
LTP  decrease na 
Gliosis Anti-GFAP staining na increase 
Expression levels of specific proteins 
 pERK decrease decrease 
 pCREB decrease decrease 
 GAD67 increase increase 
 GFAP increase increase 
Presence on Western blot of urea-soluble fraction of the brain extracts 
 Polyubiquitinated TDP-43 increase prominent increase 
 Phosphorylated TDP-43 absence absence 
 35 kD fragment of TDP-43 increase prominent increase 
 25 kD fragment of TDP-43 absence presence 
TDP-43+ NCIs adjacent to TDP-43 depleted nuclei in Tg mouse brain sections Immunohistochemistry absence presence 
Presence of TDP-43+, ubiquitin+ NCIs Immunostaining absence presence 
Hippocampus volume MRI measurement na decrease 
Neuronal loss 
 Brain weight similar to WT decrease 
 Neuronal counts similar to WT decrease 
Apoptosis TUNEL assay absence increase 
  2-mo Tg 6-mo Tg 
Cognitive functions 
 Water maze test decrease na 
 Fear conditioning task decrease na 
 Novel object recognition test decrease decrease 
Motor functions 
 Limb-clasping reflex similar to WT increase 
 Rotarod testing similar to WT decrease 
LTP  decrease na 
Gliosis Anti-GFAP staining na increase 
Expression levels of specific proteins 
 pERK decrease decrease 
 pCREB decrease decrease 
 GAD67 increase increase 
 GFAP increase increase 
Presence on Western blot of urea-soluble fraction of the brain extracts 
 Polyubiquitinated TDP-43 increase prominent increase 
 Phosphorylated TDP-43 absence absence 
 35 kD fragment of TDP-43 increase prominent increase 
 25 kD fragment of TDP-43 absence presence 
TDP-43+ NCIs adjacent to TDP-43 depleted nuclei in Tg mouse brain sections Immunohistochemistry absence presence 
Presence of TDP-43+, ubiquitin+ NCIs Immunostaining absence presence 
Hippocampus volume MRI measurement na decrease 
Neuronal loss 
 Brain weight similar to WT decrease 
 Neuronal counts similar to WT decrease 
Apoptosis TUNEL assay absence increase 

na, not analyzed.

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