Cerebral Sphingolipids in the Quaking Mouse
There are over 300 mutant genes in the mouse which are characterized by a variety of specific phenotypic expressions (Green, 1966, pp. 87–150). The “Quaking” strain of mouse has been extensively characterized genetically and histologically by Sidman, Dickie and Appel (1964) and shown to bear an autosomal recessive gene (qk) which is responsible for abnormalities in central nervous system structure and function. Affected animals can be recognized by the twelfth day pospartum by an unsteady gait and tremor of the hindquarters. Tonic-clonic seizures are induced readily by sensory stimulation in the adult Quaking animal. The principal neuropathological findings in the Quaking mice is a generalized deficiency of myelin.
KeywordsGlycogen Storage Disease Mutant Animal Metachromatic Leukodystrophy Brain Ganglioside Radioactive Precursor
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