Analysis Tools
mortality/aging
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• about 60% of mice survive postnatally; these mice have a normal life span
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growth/size/body
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• mice exhibit fewer fungiform papillae at 4 weeks, and 3, 6, and 12 months of age
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• although fungiform papillae of tongues have taste buds, they are smaller with abnormal morphology and less sensory innervation
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• aged mice are about 70% the weight of controls
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• aged mice are about 90% the length of controls
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• reduction in growth rate
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• smaller at E18.5, with shorter body lengths
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• reduced body weight at E18.5
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adipose tissue
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• mice have very little fat content
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behavior/neurological
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• mice show excessive grooming after weaning (P28)
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• hindlimb clasping that becomes severe over time
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craniofacial
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• mice exhibit fewer fungiform papillae at 4 weeks, and 3, 6, and 12 months of age
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• although fungiform papillae of tongues have taste buds, they are smaller with abnormal morphology and less sensory innervation
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digestive/alimentary system
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• mice exhibit fewer fungiform papillae at 4 weeks, and 3, 6, and 12 months of age
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• although fungiform papillae of tongues have taste buds, they are smaller with abnormal morphology and less sensory innervation
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embryo
nervous system
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• the volume of stellate ganglia is already reduced at E18.5
• however, neuronal density in stellate ganglia is not different at E18.5
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• a large fraction of superior cervical ganglia are smaller and vacuolated, with the average volume about 50% that of controls by 18 months of age
• the volume of superior cervical ganglia is already reduced at E18.5
• however, neuronal density in superior cervical is not different at E18.5, indicating postntal degeneration
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• reduction in neuronal numbers at 18 months of age
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• density of epidermal nerve fibers in the plantar hind paws of 12 month old mice is reduced, indicating reduced cutaneous sensory innervation of hind paw skin
• decrease in sensory innervation to taste buds
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• the volumes of the dorsal root ganglia are already reduced at E18.5
• the number of trkA/calcitonin gene-related peptide (CGRP) positive nociceptive neurons are reduced in the dorsal root ganglia at E18.5
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• the relative proportion of small dark B cells in lumbar dorsal root ganglia is reduced at 12 months of age while the proportion of A cells is increased
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• neurodegeneration, in particular in sympathetic ganglia, occurs mainly postnatally
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skeleton
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• the Cobb angle (formed by the intersection of two lines plotted at the end-vertebrae of the curve deformity) is increased in 6 month old mice
• however, obvious skeletal malformations are not seen at E18.5
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• slight delay in ossification is seen at E18.5, particularly a delay in ossification of parietal and intraparietal cranial bones and in the phalanges
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taste/olfaction
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• although fungiform papillae of tongues have taste buds, they are smaller with abnormal morphology and less sensory innervation
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Riley-Day syndrome | DOID:11589 |
OMIM:223900 |
J:229459 | |
