1. The Knowns and Unknowns of Prion Protein in Immune Modulation and the Pathogenesis of Neuroautoimmune Diseases.
- Author
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Abbasi Kasbi, Naghmeh and Stuve, Olaf
- Abstract
Purpose of Review: This review aims to enhance the present knowledge of PrP
C ’s role in immunological processes and to discuss how PrPC may affect immune function and contribute to the pathogenesis of neuroimmunological disorders such as MS. Recent Findings: The misfolding of the physiological cellular prion protein (PrPC ) into the pathogenic variant, scrapie prion protein (PrPSc ), underlies the pathogenesis of prion diseases. Several functions have been attributed to PrPC ; however, its actual biological role remains unclear. Systemic and local inflammation appear to be major drivers of neurodegeneration. Interestingly, PrPC is extensively found within the immune system, highlighting its significant function in modulating innate and adaptive immune responses likely playing a key role in T lymphocytes activation, differentiation and phagocytes activity. Moreover, studies involving PrPC -deficient mice in experimental autoimmune encephalomyelitis (EAE) models of the human inflammatory disorder of the central nervous system (CNS), multiple sclerosis (MS) have presented a more severe disease onset and a diminished capacity for clinical improvement. Hence, PrPC may modulate inflammation in MS and related autoimmune disorders. Summary: The lack of PrPC enhances immunological and inflammatory events, highlighting that this protein may be involved in MS development. There is still a lack of evidence assessing its role in the latest PrPC -deficient mice strain. [ABSTRACT FROM AUTHOR]- Published
- 2025
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