Altered XXXX @entity410 induction latency, phenotype, and subsequent mortality in a @entity19 model of @entity12030 .

@entity1 @entity61787 @entity410 @entity2175 @entity19 @entity73 @entity2045 @entity12030 @entity179 @entity15945 @entity70978 @entity393 @entity204
PURPOSE: @entity12030 ( @entity12030 ), or @entity12030 , is a @entity179 characterized by vision loss, @entity410 activity, @entity73 , and @entity204 . Discovery of the @entity12030 -related gene, @entity70978 , led to creation of a @entity70978 protein-deficient @entity19 model ( @entity70978 -/-), which recapitulates some of the histopathologic characteristics of the @entity1 condition. We hypothesized that lack of @entity70978 would alter @entity410 -related behavioral parameters. METHODS: Using @entity15945 gas inhalation, we examined @entity410 -induction latencies in @entity70978 -/- @entity19 and wildtype (wt) controls at time points that represent late neonatal, immature, mature, and aged time points. We examined latency to first @entity2045 (LMJ), latency to @entity393 ( @entity393 ), and subsequent mortality. RESULTS: Our results demonstrate an age-dependent alteration of @entity410 -induction latencies in @entity70978 -/-. Immature @entity61787 -/- @entity19 aged 35-42 days had an increased latency to both LMJ and @entity393 compared with age-matched wt controls. There were no significant latency differences between @entity70978 -/- and wt at other time points examined. Mortality after generalized @entity410 was high in both @entity70978 -/- and wt animals at late neonatal and immature developmental stages. No mortality was seen in wt @entity19 past @entity2175 at 6 weeks. Mature and aged @entity70978 -/- animals retained a vulnerability to death after @entity410 activity. CONCLUSIONS: These results suggest that a deficiency of @entity70978 protein in the Batten model @entity19 may result in age-dependent alteration of the neuroanatomic and biochemical substrates involved in @entity410 propagation and recovery. This may be important in understanding @entity410 , @entity179 , and @entity204 in @entity1 @entity12030 .
