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Cellular prion and its catabolites in the brain: production and function
M-V Guillot-Sestier1, F Checler
1Institut de Pharmacologie Moleculaire et Cellulaire, Equipe labellisee Fondation pour la Recherche Medicale, Sophia-Antipolis, Valbonne, France.
Current Molecular Medicine
|January 26, 2012
Summary
Cellular prion protein (PrP(c)) proteolysis yields fragments with differing functions in the nervous system. This review explores PrP(c) catabolite production and their roles in cell death and survival regulation.
Area of Science:
- Neuroscience
- Biochemistry
- Molecular Biology
Background:
- The cellular prion protein (PrP(c)) is known to convert into a pathogenic isoform (PrP(sc)) implicated in Transmissible Spongiform Encephalopathies (TSEs).
- PrP(c) is subject to proteolytic cleavage, generating distinct membrane-bound and secreted fragments.
- The functional significance of PrP(c) proteolysis, whether inactivation or maturation, remains an area of investigation.
Purpose of the Study:
- To review the mechanisms of cellular prion protein (PrP(c)) catabolite production.
- To investigate the physiological functions of PrP(c) and its fragments.
- To elucidate the role of PrP(c) and its fragments in regulating neuronal cell death and survival.
Main Methods:
- Literature review of studies on prion protein (PrP) processing and function.
- Analysis of biochemical pathways involved in PrP(c) proteolysis.
- Examination of cellular and physiological data related to PrP(c) fragments in normal and TSE-affected brains.
Main Results:
- Proteolytic processing of PrP(c) generates various fragments with distinct characteristics.
- The nature and abundance of these fragments differ between healthy and TSE-affected human brains.
- Evidence suggests PrP(c) fragments may play roles in cellular signaling pathways.
Conclusions:
- PrP(c) proteolysis is a complex process with implications for neuronal function.
- Understanding PrP(c) catabolite functions is crucial for deciphering prion diseases.
- Further research is needed to fully elucidate the physiological roles of PrP(c) fragments in cell death/survival regulation.
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