Analysis Tools
behavior/neurological
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• although mice learn the conditional stimulus, mice are unable to remember the context of the aversive stimulus in the contextual fear-conditioning test at 3 and 12 months of age
• contextual learning in the fear-conditioning paradigm is not improved by dietary intervention with a mannose-free diet
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• although mice learn the conditional stimulus, mice are unable to remember the context of the aversive stimulus in the cued fear-conditioning test at 3 and 12 months of age
• contextual learning in the fear-conditioning paradigm is not improved by dietary intervention with a mannose-free diet
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cellular
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• increase in apoptotic myofibers
• however, skeletal muscle fibrosis is not seen
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growth/size/body
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• mice show decreased body weight at 12 months, but not 3 months, of age
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homeostasis/metabolism
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• serum mannose concentrations are increased at 3 months of age
• mice fed a nominally mannose-free diet, in which mannose was replaced by sucrose, starting from P14 up to 12 months of age, show almost normalized mannose levels in 3-months of age
• however, serum levels of creatinine kinase are normal at 3 and 12 months of age
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muscle
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• increase in apoptotic myofibers
• however, skeletal muscle fibrosis is not seen
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• Z-band streaming in skeletal muscle
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• typical extracellular matrix proteins laminin, nidogen, and collagen show more fragmented localization in skeletal muscles at 12 months, but not 3 months, of age
• the connection between myofiber basal lamina and endomysial collagen is less compact and tight is skeletal muscle
• - increase in GDP-mannose levels in skeletal muscle
• the altered distribution of laminin is rescued by dietary intervention with a mannose-free diet
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• patches of myofibrillar disintegration in skeletal muscle
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• skeletal muscles show decreased myofiber diameter at 12 months of age
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• skeletal muscles show centralized nuclei at 12 months of age
• the increase in centralized nuclei is almost normalized by dietary intervention with a mannose-free diet
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• mass of M. tibialis anterior, the M. gastrocnemius together with M. soleus and the M. palmaris longus is lower at 12 months of age
• muscle mass is almost normalized by dietary intervention with a mannose-free diet
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• the foot-base angle of the hind paw at toe-off position is reduced in the beam-walk test at 3 months of age and further flattens over time, suggesting a progressive muscle weakness
• mice exhibit progressive muscle weakness in the forelimb in the weights test, holding less weight with their forepaws than wild-type mice
• aged mice fall off a wire mesh more rapidly than wild-type mice
• the deterioration of motor functions is almost completely prevented by dietary intervention with a mannose-free diet
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nervous system
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• age-dependent loss of hippocampal pyramidal neurons
• the progressive loss of hippocampal neurons and Purkinje cells is largely attenuated by dietary intervention with a mannose-free diet
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• layering of the neocortex is altered in young mice
• abnormal cortical layering is not with a mannose-free diet
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• age-dependent loss of Purkinje cells
• the progressive loss of hippocampal neurons and Purkinje cells is largely attenuated by dietary intervention with a mannose-free diet
• however, sciatic nerve fiber numbers at 12 months of age do not differ and axon diameters are normal
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• a mild decrease of the sensory nerve conduction velocity is seen in 12-month-old mice
• however, compound muscle action potentials at the tail tip in response to electrical stimulation at the root of the tail and the efferent nerve conduction velocity are not altered at 12 months of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| alacrima, achalasia, and impaired intellectual development syndrome | DOID:0112321 |
OMIM:615510 |
J:305791 | |
