UNASSIGNED: @entity64 are perhaps the most serious complications of @entity3458 ( @entity3458 ) and by the fifth decade occur in approximately 25% of @entity1 . While most @entity1 do not develop @entity64 , mounting evidence indicates that even without brain abnormalities on imaging studies, @entity3458 @entity1 can present profound @entity149 . We sought to evaluate the neurocognitive behavior profile of humanized @entity3458 @entity19 (Townes, BERK) and to identify hematologic and @entity854 associated with the behavioral alterations observed in these @entity19 . Heterozygous and homozygous Townes @entity19 displayed severe @entity73 shown by significant delays in spatial learning compared to controls. Homozygous Townes also had increased @entity308 - and @entity148 -like behaviors as well as reduced performance on voluntary wheel running compared to controls. Behavior deficits observed in Townes were also seen in BERKs. Interestingly, most deficits in homozygotes were observed in older @entity19 and were associated with worsening @entity417 . Further, @entity854 including the presence of large bands of dark/pyknotic (shrunken) neurons in @entity4778 and @entity4779 fields of hippocampus and evidence of neuronal dropout in cerebellum were present in homozygotes but not control Townes. These observations suggest that cognitive and @entity146 in @entity3458 @entity19 mirror those described in @entity3458 @entity1 and that aging, @entity417 , and profound neuropathologic changes in hippocampus and cerebellum are possible biologic correlates of those deficits. These findings support using @entity3458 @entity19 for studies of @entity73 in @entity3458 and point to vulnerable brain areas with susceptibility to @entity528 in @entity3458 and to mechanisms that potentially underlie those deficits.

XXXX in sickle cell @entity19 are associated with profound neuropathologic changes in hippocampus and cerebellum.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity64 :: ('MESH:D020521', 'Disease') :: ['strokes', 'Strokes'] @entity308 :: ('MESH:D003866', 'Disease') :: ['depression'] @entity417 :: ('MESH:D000740', 'Disease') :: ['anemia'] @entity146 :: ('MESH:D001523', 'Disease') :: ['behavioral deficits'] @entity19 :: ('10090', 'Species') :: ['mice'] @entity73 :: ('MESH:D003072', 'Disease') :: ['cognitive deficits'] @entity4779 :: ('12350(Tax:10090)', 'Gene') :: ['CA3'] @entity149 :: ('MESH:D002493', 'Disease') :: ['neurocognitive dysfunction'] @entity3458 :: ('MESH:D000755', 'Disease') :: ['SCD', 'sickle cell disease'] @entity4778 :: ('12346(Tax:10090)', 'Gene') :: ['CA1'] @entity854 :: ('MESH:D018376', 'Disease') :: ['neuropathologic abnormalities'] @entity148 :: ('MESH:D001008', 'Disease') :: ['anxiety'] @entity528 :: ('MESH:D009410', 'Disease') :: ['neuronal injury']
@entity73 :: (MESH:D003072,Disease) :: ['Cognitive and behavior deficits']
