Analysis Tools
muscle
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• at 65 weeks of age, degenerated muscle is almost entirely replaced by fatty tissue in areas of the posterior tongue muscle
• only a few muscle cells remain but appear highly abnormal or vacuolated amid a field of fatty tissue
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• at 65 weeks of age, mutant axial skeletal muscles display degenerative processes, including migration of nuclei into the sarcoplasm, hyalin degeneration of the sarcoplasm, vacuolization of the sarcoplasm, infiltration of macrophages, fibrosis, and fatty replacement of muscle cells
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• homozygotes exhibit chronic muscular dystrophy, with only very minimal axial muscular dystrophy noted at 7 weeks of age
• by 20 weeks of age, severely dystrophic axial muscles are associated with occasional fibrosis, fatty infiltration, and myositis and include muscles of the head while the diaphragm and some hindlimb muscles (e.g. soleus) are only mildly affected
• by 40 weeks of age, axial muscular dystrophy is more severe, fibrosis and fatty infiltrates are pronounced, and muscles of the head exhibit degeneration while the soleus muscle is still moderately affected
• at all ages, the most severely affected muscle cells are generally located adjacent to bone or at tendinous insertions and less frequently deep within muscle bundles
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• at 65 weeks of age, hunched homozygotes show severe degeneration of some skeletal muscles, including the muscles of posterior tongue, head (particularly those of the hyoid apparatus), and surrounding the axial skeleton
• at this age, limb muscles are affected to a lesser extent while the heart muscle appears normal
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• aging homozygotes exhibit progressive muscle weakness in the tongue, head regions, and muscles associated with the axial skeleton
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skeleton
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• hunched homozygotes exhibit accelerated degenerative osteoarthritis of the intervertebral facet joints
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• at 12 months of age, homozygotes display a severe curvature of the spine (anterior kyphosis) and emaciation
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growth/size/body
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• at 65 weeks of age, degenerated muscle is almost entirely replaced by fatty tissue in areas of the posterior tongue muscle
• only a few muscle cells remain but appear highly abnormal or vacuolated amid a field of fatty tissue
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weight loss
(
J:72926
)
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• homozygotes consistently start losing weight after ~40 weeks of age
• in aging homozygotes, progressive loss of weight and body fat is probably due to an impaired ability to obtain nourishment as a result of muscle weakness in the tongue and head regions
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behavior/neurological
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• at 40 weeks of age, homozygotes begin to display a hunched posture due to kyphosis
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adipose tissue
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• homozygotes eventually lose most of their body fat
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vision/eye
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• at 11 weeks of age, homozygotes show a 7% reduction in average lens mass relative to wild-type mice
• however, mutant lenses remain as transparent as wild-type lenses, with no evidence of cytoplasmic inclusion bodies in the lens nuclei, and no significant differences in histology and equatorial and axial dimensions relative to wild-type lenses
• in addition, the thermal stability of a lens homogenate supernatant is only mildly compromised, and mutant lenses show no differences in loss of lens transparency when oxidatively stressed in vivo with hyperbaric oxygen relative to wild-type lenses
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immune system
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• hunched homozygotes exhibit accelerated degenerative osteoarthritis of the intervertebral facet joints
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craniofacial
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• at 65 weeks of age, degenerated muscle is almost entirely replaced by fatty tissue in areas of the posterior tongue muscle
• only a few muscle cells remain but appear highly abnormal or vacuolated amid a field of fatty tissue
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digestive/alimentary system
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• at 65 weeks of age, degenerated muscle is almost entirely replaced by fatty tissue in areas of the posterior tongue muscle
• only a few muscle cells remain but appear highly abnormal or vacuolated amid a field of fatty tissue
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nervous system
| N |
• mice exhibit normal dopaminergic neuron numbers
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astrocytosis
(
J:194407
)
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• in the substantia nigra
• not suppressed by quinpirole
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