Analysis Tools
mortality/aging
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• mortality resulting from starvation occurs, with all mice succumbing by ~200 days, with mean survival of 118 days
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growth/size/body
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• by 80 days of age, mice weigh 45% less than wild-type and 30% less than single homozgyotes by 80 days of age
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nervous system
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• DRG cell bodies from which the gracile axons emanate show increased degeneration, with smaller cell diameter and more basophilic cytoplasm; abnormal cell morphology is 4.1-fold higher than in wild-type
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• more severe than Uchl1gad homozygotes
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• at ~90 days of age, dystrophic axons or spheroids are observed in sections of gracile nucleus
• increased numbers of spheroids are seen in double mutants compared to Uchl-null mice in nucleus tractus solitarus and area postrema; increase is seen in gracile nucleus of the medulla and in the gracile fascicle of the spinal cord at cervical and thoracic levels
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behavior/neurological
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• occurs with high penetrance compared to single homozygotes; associated with terminal stages and range from several days to several weeks in duration
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abnormal gait
(
J:71819
)
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• both hindlimb digits and footpads make contact when walking ('flatfooted'), whereas only digits make contact when wild-type mice walk
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• at time of death, mice display only moderate posterior paralysis compared to more severely affected age-matched Uchl1gad mutants
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digestive/alimentary system
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• occurs with high penetrance compared to single homozygotes; associated with terminal stages and range from several days to several weeks in duration
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