mortality/aging
• all die by 4 weeks of age
• twice daily treatment with 50 mg/kg L-dihydroxyphenylalanine (L-DOPA) starting around P15 rescues the lethality
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growth/size/body
• at P10 - P15
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• at P16, body weight is 70% of control littermates
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weight loss
(
J:30404
)
• after P17 a negative growth rate is seen
• twice daily treatment with 50 mg/kg L-DOPA starting around P15 restores a nearly normal growth rate
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behavior/neurological
• injection with L-DOPA restores eating behavior with maximal intake during the first 2 hours after injection and cessation of eating by about 6 hours post injection
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poor grooming
(
J:30404
)
• at P10 - P15
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• at P10 - P15
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• at P10 - P15, travel only about 3m/hr compared to 23 m/hr in controls
• however at P16, reflexion, corneal, startle, righting, grasping, and placing reflexes are all similar to controls, balance is not impaired, and no tremors are seen
• within 15 minutes of injection of L-DOPA activity levels increase peaking at about 1 hour post injection and then decreasing so that by 12 hours post injection mice are hypoactive
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• make about 280 stereotypical beam breaks per hour compared to about 700 per hour for controls
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nervous system
• brain size is reduced and neurons are slightly more compact; however, dopaminergic neuron and striatum morphology are similar to controls
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• expression levels of tachykinin 1 and dynorphin are decreased in the striatum
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homeostasis/metabolism
• dopamine levels in the brain are low; however, levels of norepinephrine in the brain, heart, salivary glands, and adrenal glands are similar to controls
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