mortality/aging
• died approximately 30 days after birth
|
behavior/neurological
limb grasping
(
J:94227
)
• retracted their hind feet and clenched their digits when suspended by tail
|
• decreased performance in an inclined-platform test with fewer mice reaching the top and taking longer to climb to the top of the platform, however, 63% of mutants remained on the platform suggesting that muscle strength is not impaired
|
• characterized by a progressive movement disorder beginning at P18
|
• decreased mobility
|
cardiovascular system
small heart
(
J:94227
)
cellular
• only mutant MEFs contained abnormal mitochondria and the increase in the number of abnormal mitochondria following mitochondrial stress was greater in mutant MEFs, indicating increased sensitivity to stress
|
• increased sensitivity to apoptosis induction by DNA-damaging agent etoposide
|
• significant increase in the susceptibility of cultured primary cortical neurons to excitotoxic stimuli
|
growth/size/body
• runted mice showed a general decrease in organ size
|
• failed to gain weight following weaning at P18
|
hematopoietic system
small thymus
(
J:94227
)
small spleen
(
J:94227
)
immune system
small thymus
(
J:94227
)
small spleen
(
J:94227
)
nervous system
• significant increase in the susceptibility of cultured primary cortical neurons to excitotoxic stimuli
|
• at P30, brain was approximately 75% of the weight of wild-type
|
• extracts from striata showed a significant decrease in the yield of mitochondrial citrate synthase suggesting decreased mitochondrial density
|
• at P20 and P30, observed selective loss of a population of striatal neurons, just lateral of the thalamus in a posteriomedial portion of the basal ganglia, and loss of terminals of the nigrostriatal pathway
|
homeostasis/metabolism
• significant increase in the susceptibility of cultured primary cortical neurons to excitotoxic stimuli
|
integument
sparse hair
(
J:94227
)
• hair loss was occasionally seen in mutants after P18
|
endocrine/exocrine glands
small thymus
(
J:94227
)