Analysis Tools
behavior/neurological
|
• mice show impaired performance on the fixed and accelerating rotarod at 8 to 12 months of age, but not at 3 months, with a lower latency to fall at fixed speed and falling off the accelerating rod at a lower speed than controls
• however, mice exhibit normal hind limb coordination in walking across a notched bar
|
|
• mice show increased grip force immediately prior to releasing its grasp
|
|
• mice are only able to run about half of the distance covered by wild-type mice on a treadmill, indicating susceptibility to fatigue and decreased endurance to exercise
• however, mice travel a similar distance as wild-type mice in the open field
|
cellular
|
• decrease in mitochondrial DNA content in muscles
|
|
• decrease in oxidative state and capacity of muscle
• while mitochondrial respiration rates during state 3 respiration with ADP is similar to wild-type, they are higher in both states 2 (basal respiration in the presence of substrate alone) and 4o (respiration in presence of oligomycin, an inhibitor of ATPase)
• however, the respiratory control ratio remains unchanged
|
|
• skeletal muscle mitochondria show a slight increase in respiration during non-phosphorylating conditions and a shift in the relationship between oxygen consumption and mitochondrial membrane potential indicative of a greater proton leak
• mitochondria exhibit a higher rate of oxygen consumption, a slight change in the inner membrane proton conductance, and increase in membrane permeability
|
homeostasis/metabolism
|
• mice are only able to run about half of the distance covered by wild-type mice on a treadmill, indicating susceptibility to fatigue and decreased endurance to exercise
• however, mice travel a similar distance as wild-type mice in the open field
|
muscle
|
• decrease in mitochondrial DNA content in muscles
|
|
• in the tibialis anterioris, some fibers (less than 10%) have few small-sized mitochondria
|
|
• a decrease in proportion of type I slow-twitch myofibers in the soleus
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Down syndrome | DOID:14250 |
OMIM:190685 |
J:223518 | |
