Analysis Tools
behavior/neurological
| N |
• at 3 months, no defects observed in an open field test, a rotarod test, a hanging wire test or a hot plate test
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• in mice greater than 8 weeks of age, about 7% had generalized, tonic-clonic seizure
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skeleton
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• reduction in the size of the intervertebral foramina
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• after 6 months of age, kyphoscoliotic malformations of lower thoracic and lumbar vertebral column
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• vertebral arches have stunted pediculi that extend over almost the entire length of the vertebral bodies at 15 months of age
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• at 15 months of age, vertebral bodies are thickened along their dorsoventral axis and show a reduced craniocaudal height
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• reduction in calcium salts within compact bone
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• regions of cancellous bone, most notably within vertebral bodies, show an abnormally coarse and locally hyperdense meshwork of mostly longitudinally oriented trabeculations
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• decreased mineralization, beginning at 6 months of age and progressive
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nervous system
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• in mice greater than 8 weeks of age, about 7% had generalized, tonic-clonic seizure
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• microglial morphology shows a change toward an epithelioid morphology at later ages
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astrocytosis
(
J:41986
)
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• localized astrogliosis in those regions showing increased lamp-1 staining
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cellular
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• lysosomal storage is observed in the kidney (podocytes and distinct segments of the nephron) and in the brain (astroglia, cerebellar Bergmann glia, ependymal cells, and microglial cells)
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• enlarged lysosomes in some podocytes and portions of Henle's loop that are located within the inner stripe of the outer medulla
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renal/urinary system
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• enlarged lysosomes in some podocytes and portions of Henle's loop that are located within the inner stripe of the outer medulla
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hematopoietic system
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• microglial morphology shows a change toward an epithelioid morphology at later ages
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immune system
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• microglial morphology shows a change toward an epithelioid morphology at later ages
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