The cellular prion protein ( @entity3669 ) is a membrane-bound glycoprotein mainly present in the CNS. The scrapie prion protein (PrP(Sc)) is an isoform of @entity3669 , and it is responsible for @entity1579 ( @entity1579 ), a group of @entity179 affecting both @entity1 and animals. The presence of the cellular form is necessary for the establishment and further evolution of @entity1579 . Here, we map the regional distribution of @entity3669 in the @entity35 brain and study the chemical nature of these immunopositive neurons. Our observations are congruent with retrograde transport of prions, as shown by the ubiquitous distribution of @entity3669 throughout the @entity35 brain, but especially in the damaged areas that send projections to primarily affected nuclei in @entity14749 . On the other hand, the presence of the cellular isoform in a subset of GABAergic neurons containing @entity460 -binding proteins suggests that @entity3669 plays a role in the metabolism of @entity460 . The lack of immunostaining in neurons ensheathed by perineuronal nets indicates that prions do not directly interact with components of these nets. The destruction of these nets is more likely to be the consequence of a factor needed for prions during the early stages of @entity1579 . This would cause destruction of these nets and death of the surrounded neurons. Our results support the view that destruction of this extracellular matrix is caused by the pathogenic effect of prions and not a primary event in @entity1579 .

Expression of @entity3669 in the XXXX brain and characterization of a subset of cortical neurons.

@entity1 :: ('9606', 'Species') :: ['humans'] @entity1579 :: ('MESH:D017096', 'Disease') :: ['TSEs', 'prion diseases', 'transmissible spongiform encephalopathies'] @entity3669 :: ('5621', 'Gene') :: ['PrP(C)'] @entity179 :: ('MESH:D019636', 'Disease') :: ['neurodegenerative diseases'] @entity460 :: ('MESH:D002118', 'Chemical') :: ['calcium'] @entity35 :: ('10116', 'Species') :: ['rat'] @entity14749 :: ('MESH:D034062', 'Disease') :: ['fatal familial insomnia']
@entity35 :: (10116,Species) :: ['rat']
