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Reelin Expression in Creutzfeldt-Jakob Disease and Experimental Models of Transmissible Spongiform Encephalopathies.

Abstract：

:Reelin is an extracellular glycoprotein involved in key cellular processes in developing and adult nervous system, including regulation of neuronal migration, synapse formation, and plasticity. Most of these roles are mediated by the intracellular phosphorylation of disabled-1 (Dab1), an intracellular adaptor molecule, in turn mediated by binding Reelin to its receptors. Altered expression and glycosylation patterns of Reelin in cerebrospinal and cortical extracts have been reported in Alzheimer's disease. However, putative changes in Reelin are not described in natural prionopathies or experimental models of prion infection or toxicity. With this is mind, in the present study, we determined that Reelin protein and mRNA levels increased in CJD human samples and in mouse models of human prion disease in contrast to murine models of prion infection. However, changes in Reelin expression appeared only at late terminal stages of the disease, which prevent their use as an efficient diagnostic biomarker. In addition, increased Reelin in CJD and in in vitro models does not correlate with Dab1 phosphorylation, indicating failure in its intracellular signaling. Overall, these findings widen our understanding of the putative changes of Reelin in neurodegeneration.

journal_name

Mol Neurobiol

journal_title

Molecular neurobiology

authors

Mata A,Urrea L,Vilches S,Llorens F,Thüne K,Espinosa JC,Andréoletti O,Sevillano AM,Torres JM,Requena JR,Zerr I,Ferrer I,Gavín R,Del Río JA

doi

10.1007/s12035-016-0177-8

subject

Has Abstract

pub_date

2017-10-01 00:00:00

pages

6412-6425

issue

8

eissn

0893-7648

issn

1559-1182

pii

10.1007/s12035-016-0177-8

journal_volume

54

pub_type

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