behavior/neurological
• mice exhibit an ataxic gait and flick their tails in a rapid side-to-side movement beginning at 5-6 months of age
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• 5-6 month old mice are able to rear on hind legs, but typically topple over after a few seconds
• mice are unable to walk along a balance beam and immediately swing perpendicular to the beam, hanging by the forepaws and falling off within a few seconds
• mice exhibit a normal grip strength and righting reflex
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hematopoietic system
• reduction in red blood cell volume
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• reduction in hemoglobin levels
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homeostasis/metabolism
• fasting plasma glucose levels are slightly reduced on a chow diet, but this difference is not seen in mice fed a high-fat diet
• however, mice do not have altered glucose tolerance
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• on a high-fat diet, plasma triglyceride and free fatty acid levels are 20% lower in mutants
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• on a high-fat diet, plasma triglyceride and free fatty acid levels are 20% lower in mutants
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• on a high-fat diet, mutants accumulate more hepatic triacylglycerol stores than wild-type mice
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• phosphatidate phosphatase (PAP) enzyme activity in the liver is increased, while activity in white adipose tissue and skeletal muscle is normal
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liver/biliary system
• on a high-fat diet, mutants accumulate more hepatic triacylglycerol stores than wild-type mice
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nervous system
• ataxic mice show a 50% increase in total phosphatidic acid levels (a PAP enzyme substrate) in the cerebellum
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• the Purkinje cell layer exhibits an irregular appearance in 10 month old mutants
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immune system
N |
• mice do not develop osteomyelitis with age
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