The Quest for Cellular Prion Protein Functions in the Aged and Neurodegenerating Brain

Cells

Isidre Ferrer¹  Laia Lidón²  Rosalina Gavín²  JoséAntonio del Río² 

[1] Center for Networked Biomedical Research on Neurodegenerative Diseases (Ciberned), 28031 Barcelona, Spain;Molecular and Cellular Neurobiotechnology, Institute for Bioengineering of Catalonia (IBEC), Barcelona Institute of Science and Technology (BIST), Science Park of Barcelona, 08028 Barcelona, Spain;

关键词: prion;    tau;    alzheimer’s disease;    parkinson’s disease;    huntington’s disease;    neuroprotection;   

DOI  :  10.3390/cells9030591

来源: DOAJ

【 摘 要 】

Cellular (also termed ‘natural’) prion protein has been extensively studied for many years for its pathogenic role in prionopathies after misfolding. However, neuroprotective properties of the protein have been demonstrated under various scenarios. In this line, the involvement of the cellular prion protein in neurodegenerative diseases other than prionopathies continues to be widely debated by the scientific community. In fact, studies on knock-out mice show a vast range of physiological functions for the protein that can be supported by its ability as a cell surface scaffold protein. In this review, we first summarize the most commonly described roles of cellular prion protein in neuroprotection, including antioxidant and antiapoptotic activities and modulation of glutamate receptors. Second, in light of recently described interaction between cellular prion protein and some amyloid misfolded proteins, we will also discuss the molecular mechanisms potentially involved in protection against neurodegeneration in pathologies such as Alzheimer’s, Parkinson’s, and Huntington’s diseases.

【 授权许可】

Unknown