@entity14697 ( @entity14697 ) is a segmental @entity43608 with multiple features suggestive of premature accelerated aging. Accumulation of progerin is thought to underlie the pathophysiology of @entity14697 . However, despite ubiquitous expression of @entity31330 in all differentiated cells, the @entity14697 mutation results in organ-specific defects. For example, bone and skin are strongly affected by @entity14697 , while the brain appears to be unaffected. There are no definite explanations as to the variable sensitivity to progeria disease among different organs. In addition, low levels of progerin have also been found in several tissues from normal individuals, but it is not clear if low levels of progerin contribute to the aging of the brain. In an attempt to clarify the origin of this phenomenon, we have developed an inducible transgenic @entity19 model with expression of the most common @entity14697 mutation in brain, skin, bone and heart to investigate how the mutation affects these organs. Ultrastructural analysis of neuronal nuclei after 70 weeks of expression of the @entity31330 @entity47668 mutation showed severe distortion with multiple lobulations and irregular extensions. Despite severe distortions in the nuclei of hippocampal neurons of @entity14697 animals, there were only negligible changes in gene expression after 63 weeks of transgenic expression. Behavioral analysis and neurogenesis assays, following long-term expression of the @entity14697 mutation, did not reveal significant pathology. Our results suggest that certain tissues are protected from functional deleterious effects of progerin.

Expression of progerin in aging XXXX brains reveals structural nuclear abnormalities without detectible significant alterations in gene expression, hippocampal stem cells or behavior.

@entity31330 :: ('16905(Tax:10090)', 'Gene') :: ['LMNA', 'lamin A'] @entity19 :: ('10090', 'Species') :: ['mouse'] @entity43608 :: ('MESH:C536423', 'Disease') :: ['progeroid syndrome'] @entity47668 :: ('c|SUB|C|1824|T', 'DNAMutation') :: ['c.1824C>T'] @entity14697 :: ('MESH:D011371', 'Disease') :: ['HGPS', 'Hutchinson-Gilford progeria syndrome']
@entity19 :: (10090,Species) :: ['mouse']
