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

被引:11
作者
Gavin, Rosalina [1 ,2 ,3 ,4 ]
Lidon, Laia [1 ,2 ,3 ,4 ]
Ferrer, Isidre [3 ,4 ,5 ,6 ,7 ]
Antonio del Rio, Jose [1 ,2 ,3 ,4 ]
机构
[1] Barcelona Inst Sci & Technol, Inst Bioengn Catalonia IBEC, Mol & Cellular Neurobiotechnol, Sci Pk Barcelona, Barcelona 08028, Spain
[2] Univ Barcelona, Fac Biol, Dept Cell Biol Physiol & Immunol, E-08028 Barcelona, Spain
[3] Ctr Networked Biomed Res Neurodegenerat Dis Ciber, Barcelona 28031, Spain
[4] Univ Barcelona, Inst Neurosci, E-08028 Barcelona, Spain
[5] Univ Barcelona, Dept Pathol & Expt Therapeut, Barcelona 08907, Spain
[6] Bellvitge Univ Hosp, Barcelona 08907, Spain
[7] Bellvitge Biomed Res Inst IDIBELL, Barcelona 08908, Spain
关键词
prion; Tau; Alzheimer's disease; Parkinson's disease; Huntington's disease; neuroprotection; AMYLOID-BETA OLIGOMERS; METABOTROPIC GLUTAMATE RECEPTORS; PATHOLOGICAL ALPHA-SYNUCLEIN; STRAUSSLER-SCHEINKER DISEASE; STRESS-INDUCIBLE PROTEIN-1; CREUTZFELDT-JAKOB-DISEASE; TO-NEURON TRANSMISSION; N-TERMINAL FRAGMENT; LEWY-BODY-DISEASE; A-BETA;
D O I
10.3390/cells9030591
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
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.
引用
收藏
页数:24
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