title: The Unexposed Secrets of Prion Protein Oligomers.
abstract: According to the "protein-only" hypothesis, the misfolding and conversion of host-derived cellular prion protein (PrP(C)) into pathogenically misfolded PrP are believed to be the key procedure in the pathogenesis of prion diseases. Intermediate, soluble oligomeric prion protein (PrP) aggregates were considered a critical process for prion diseases. Several independent studies on PrP oligomers gained insights into oligomers' formation, biophysical and biochemical characteristics, structure conversion, and neurotoxicity. PrP oligomers are rich in β-sheet structure and slightly resistant to proteinase K digestion. PrP oligomers exhibited more neurotoxicity and induced neuronal apoptosis in vivo and/or in vitro. In this review, we summarized recent studies regarding PrP oligomers and the relationship between misfolded PrP aggregates and neuronal death in the course of prion diseases.
authors.affiliation: Department of Bioengineering, Huanghuai University, 463000, Zhumadian, China. wgl939@126.com.
authors.forename: Gailing
authors.initials: G
authors.lastname: Wang
authors.affiliation: Department of Bioengineering, Huanghuai University, 463000, Zhumadian, China.
authors.forename: Mingcheng
authors.initials: M
authors.lastname: Wang
authors.affiliation: Department of Bioengineering, Huanghuai University, 463000, Zhumadian, China.
authors.forename: Chuanfeng
authors.initials: C
authors.lastname: Li
mesh_terms: D000818:Animals; D006801:Humans; D017096:Prion Diseases / Q000378:metabolism*; D011328:Prions / Q000737:chemistry / Q000378:metabolism*; D066263:Protein Aggregation, Pathological / Q000378:metabolism*; D017510:Protein Folding
chemical_list: D011328:Prions
references.citation: Acta Biochim Biophys Sin (Shanghai). 2013 Jun;45(6):494-502
references.pmid: 23459558
references.citation: Biochem J. 2008 Dec 1;416(2):297-305
references.pmid: 18684106
references.citation: Science. 2010 Feb 26;327(5969):1132-5
references.pmid: 20110469
references.citation: Science. 2007 Dec 21;318(5858):1900-3
references.pmid: 18096801
references.citation: Cell. 1993 Jul 2;73(7):1339-47
references.pmid: 8100741
references.citation: Hum Mol Genet. 2012 Mar 1;21(5):1138-44
references.pmid: 22100763
references.citation: Amyloid. 2005 Jun;12(2):88-95
references.pmid: 16011984
references.citation: Nature. 1996 Jan 25;379(6563):339-43
references.pmid: 8552188
references.citation: Nature. 2000 Jan 6;403(6765):98-103
references.pmid: 10638761
references.citation: Acta Biochim Biophys Sin (Shanghai). 2013 Jun;45(6):452-64
references.pmid: 23439666
references.citation: EMBO J. 2008 Jul 23;27(14):1974-84
references.pmid: 18566584
references.citation: Acta Biochim Biophys Sin (Shanghai). 2013 Nov;45(11):899-903
references.pmid: 24041958
references.citation: Biochemistry. 2000 Jan 18;39(2):424-31
references.pmid: 10631004
references.citation: J Biol Chem. 1995 Feb 17;270(7):3299-305
references.pmid: 7852415
references.citation: Nature. 1993 Apr 8;362(6420):543-6
references.pmid: 8464494
references.citation: Biochemistry. 2005 Dec 20;44(50):16649-57
references.pmid: 16342955
references.citation: FASEB J. 2007 Oct;21(12):3107-17
references.pmid: 17494993
references.citation: J Mol Biol. 2008 Feb 15;376(2):582-96
references.pmid: 18158160
references.citation: Science. 1997 Jan 17;275(5298):402-5
references.pmid: 8994041
references.citation: Brain Pathol. 1995 Jan;5(1):43-51
references.pmid: 7767490
references.citation: J Biol Chem. 2004 Jul 23;279(30):31374-82
references.pmid: 15133040
references.citation: Nature. 1996 Jul 11;382(6587):180-2
references.pmid: 8700211
references.citation: Nat Med. 1998 Oct;4(10):1157-65
references.pmid: 9771749
references.citation: J Mol Biol. 2005 Apr 1;347(3):665-79
references.pmid: 15755458
references.citation: Biochemistry. 2001 Jun 12;40(23):6982-7
references.pmid: 11389614
references.citation: J Mol Neurosci. 2015 May;56(1):131-44
references.pmid: 25479824
references.citation: Proc Natl Acad Sci U S A. 1998 Nov 10;95(23):13363-83
references.pmid: 9811807
references.citation: PLoS One. 2014 Jun 03;9(6):e98753
references.pmid: 24892647
references.citation: Int J Alzheimers Dis. 2011 Feb 08;2011:906964
references.pmid: 21331330
references.citation: Trends Neurosci. 2012 Feb;35(2):92-103
references.pmid: 22137337
references.citation: Prog Mol Biol Transl Sci. 2012;107:101-24
references.pmid: 22482449
references.citation: Neural Regen Res. 2013 Oct 25;8(30):2868-78
references.pmid: 25206608
references.citation: J Biol Chem. 2002 Jun 14;277(24):21140-8
references.pmid: 11912192
references.citation: Trends Mol Med. 2011 Jan;17(1):14-24
references.pmid: 20889378
references.citation: Biochem Biophys Res Commun. 2005 Mar 4;328(1):292-305
references.pmid: 15670783
references.citation: Nature. 2009 Feb 26;457(7233):1128-32
references.pmid: 19242475
references.citation: Nat Rev Microbiol. 2004 Nov;2(11):861-71
references.pmid: 15494743
references.citation: J Mol Biol. 2005 Feb 18;346(2):645-59
references.pmid: 15670611
references.citation: J Pineal Res. 2014 Nov;57(4):427-34
references.pmid: 25251028
references.citation: Proc Natl Acad Sci U S A. 1993 Dec 1;90(23):10962-6
references.pmid: 7902575
references.citation: Neurobiol Dis. 2000 Aug;7(4):299-309
references.pmid: 10964602
references.citation: J Biol Chem. 2006 May 12;281(19):13828-36
references.pmid: 16554307
references.citation: FASEB J. 2010 Sep;24(9):3536-43
references.pmid: 20400537
references.citation: Mol Neurobiol. 2014 Apr;49(2):1102-16
references.pmid: 24510275
references.citation: PLoS Pathog. 2007 Aug 31;3(8):e125
references.pmid: 17784787
references.citation: J Mol Biol. 2006 Sep 29;362(4):810-20
references.pmid: 16949096
references.citation: Neurobiol Dis. 2009 Sep;35(3):352-8
references.pmid: 19523517
references.citation: Int J Dev Neurosci. 2000 Jul-Aug;18(4-5):481-92
references.pmid: 10817932
references.citation: J Biol Chem. 2001 Nov 23;276(47):43516-23
references.pmid: 11533027
references.citation: Protein Sci. 1993 Dec;2(12):2206-16
references.pmid: 7905316
references.citation: Science. 2012 Mar 9;335(6073):1228-31
references.pmid: 22403391
references.citation: Nature. 1996 Mar 28;380(6572):345-7
references.pmid: 8598929
references.citation: Biophys J. 2011 Sep 21;101(6):1483-92
references.pmid: 21943430
references.citation: J Biol Chem. 2000 Dec 1;275(48):38081-7
references.pmid: 10967124
references.citation: J Neurochem. 1999 Oct;73(4):1557-65
references.pmid: 10501201
references.citation: Science. 1982 Apr 9;216(4542):136-44
references.pmid: 6801762
references.citation: Nat Commun. 2013;4:2416
references.pmid: 24022506
references.citation: EMBO J. 1999 Dec 1;18(23):6855-64
references.pmid: 10581259
references.citation: Annu Rev Neurosci. 2003;26:267-98
references.pmid: 12704221
references.citation: Science. 1997 Jul 4;277(5322):94-8
references.pmid: 9204907
references.citation: Oncogene. 2003 Nov 24;22(53):8543-67
references.pmid: 14634618
references.citation: FEBS Lett. 1997 Aug 18;413(2):277-81
references.pmid: 9280297
references.citation: CNS Neurosci Ther. 2014 Aug;20(8):737-47
references.pmid: 24629137
references.citation: Biochem Biophys Res Commun. 2005 Feb 18;327(3):894-9
references.pmid: 15649429
references.citation: J Biol Chem. 2010 May 7;285(19):14083-7
references.pmid: 20304915
references.citation: Anal Biochem. 2012 Jul 1;426(1):54-62
references.pmid: 22490465
references.citation: Acta Biochim Biophys Sin (Shanghai). 2014 Jul;46(7):531-9
references.pmid: 24829398