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2017-10-01

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Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/141772

Reelin expression in Creutzfeldt-Jakob disease and experimental models of transmissible spongiform encephalopathies

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https://doi.org/10.1007/s12035-016-0177-8

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.

Subject

Metabolisme, Malaltia de Creutzfeldt-Jakob, Matriu extracel·lular, Teixit nerviós, Malalties per prions

Subject (English)

Metabolism, Creutzfeldt-Jakob disease, Extracellular matrix, Nerve tissue, Prion diseases

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Articles publicats en revistes (Patologia i Terapèutica Experimental)
Articles publicats en revistes (Biologia Cel·lular, Fisiologia i Immunologia)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Institut de Bioenginyeria de Catalunya (IBEC))

Citation

MATA RODRÍGUEZ, Agata, et al. Reelin expression in Creutzfeldt-Jakob disease and experimental models of transmissible spongiform encephalopathies. Molecular Neurobiology. 2017. Vol. 54, num. 8, pags. 6412-6425. ISSN 0893-7648. [consulted: 14 of May of 2026]. Available at: https://hdl.handle.net/2445/141772

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