Protein inheritance (prions) based on parallel in-register beta-sheet amyloid structures.
Most prions (infectious proteins) are self-propagating amyloids (filamentous protein multimers), and have been found in both mammals and fungal species. The prions [URE3] and [PSI+] of yeast are disease agents of Saccharomyces cerevisiae while [Het-s] of Podospora anserina may serve a normal cellular function. The parallel in-register beta-sheet structure shown by prion amyloids makes possible a templating action at the end of filaments which explains the faithful transmission of variant differences in these molecules. This property of self-reproduction, in turn, allows these proteins to act as de facto genes, encoding heritable information.
Wickner
Reed B
RB
Laboratory of Biochemistry and Genetics, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892-0830, USA. wickner@helix.nih.gov
Shewmaker
Frank
F
Kryndushkin
Dmitry
D
Edskes
Herman K
HK
D000682:Amyloid / Q000737:chemistry* / Q000235:genetics / Q000648:ultrastructure; D005979:Glutathione Peroxidase; D010454:Peptide Termination Factors; D048468:Podospora / Q000737:chemistry* / Q000235:genetics; D011328:Prions / Q000737:chemistry* / Q000235:genetics / Q000648:ultrastructure; D017433:Protein Structure, Secondary; D012441:Saccharomyces cerevisiae / Q000737:chemistry* / Q000235:genetics; D029701:Saccharomyces cerevisiae Proteins / Q000737:chemistry* / Q000235:genetics / Q000648:ultrastructure
D000682:Amyloid; D010454:Peptide Termination Factors; D011328:Prions; C068068:SUP35 protein, S cerevisiae; D029701:Saccharomyces cerevisiae Proteins; D005979:Glutathione Peroxidase; C067020:URE2 protein, S cerevisiae
Cell. 1998 Jun 26;93(7):1241-52
9657156
Proc Natl Acad Sci U S A. 2002 Dec 10;99 Suppl 4:16446-53
12461168
Nature. 2004 Mar 18;428(6980):319-23
15029195
Genetics. 2005 Mar;169(3):1227-42
15545639
Proc Natl Acad Sci U S A. 1997 Nov 11;94(23):12503-8
9356479
FEMS Microbiol Rev. 2002 Aug;26(3):223-38
12165425
Yeast. 1988 Sep;4(3):159-78
3059716
Proc Natl Acad Sci U S A. 2003 May 27;100(11):6616-21
12719532
Nature. 1994 Aug 11;370(6489):471-4
7913989
Proc Natl Acad Sci U S A. 2002 Dec 24;99(26):16742-7
12481027
Science. 1982 Dec 24;218(4579):1309-11
6815801
Mol Biol Cell. 2007 Dec;18(12):4803-12
17881723
Nature. 1967 May 20;214(5090):764-6
4963878
Nature. 1967 Sep 2;215(5105):1043-4
4964084
Cell. 2005 Apr 22;121(2):195-206
15851027
Proc Natl Acad Sci U S A. 2002 Dec 10;99 Suppl 4:16392-9
12149514
Genetics. 1996 Dec;144(4):1375-86
8978027
Science. 2008 Mar 14;319(5869):1523-6
18339938
J Mol Biol. 1988 Feb 20;199(4):559-73
3280807
Mol Cell Biol. 2004 May;24(9):3928-37
15082786
Mol Gen Genet. 1975;136(4):327-35
16095000
Acc Chem Res. 2005 Dec;38(12):911-21
16359163
EMBO J. 1995 Aug 15;14(16):4065-72
7664746
Science. 2003 Apr 25;300(5619):640-3
12690204
Mol Cell Biol. 1988 Dec;8(12):5528-40
3149717
Biochemistry. 2007 Nov 13;46(45):13149-62
17953455
Biochemistry. 1991 Oct 29;30(43):10382-7
1931962
Nature. 2007 May 24;447(7143):453-7
17468747
J Bacteriol. 1972 Jan;109(1):203-8
4550662
Genetics. 1994 Jul;137(3):671-6
8088512
EMBO J. 2005 Sep 7;24(17):3082-92
16096644
Proc Natl Acad Sci U S A. 2006 Dec 26;103(52):19754-9
17170131
Nature. 2005 Jun 9;435(7043):773-8
15944695
J Mol Biol. 2007 Jan 19;365(3):773-82
17097676
Proc Natl Acad Sci U S A. 2004 Jan 20;101(3):711-6
14715898
Mol Cell Biol. 1999 Dec;19(12):8103-12
10567536
J Biol Chem. 2007 Apr 20;282(16):11931-40
17324933
J Virol. 1992 Apr;66(4):2096-101
1347795
Proc Natl Acad Sci U S A. 2008 Sep 9;105(36):13345-50
18757753
Mol Cell Biol. 2006 Jan;26(2):617-29
16382152
Proc Natl Acad Sci U S A. 1998 Nov 10;95(23):13407-12
9811813
BMC Mol Biol. 2006 Oct 11;7:34
17034622
Genetics. 2007 Jul;176(3):1557-65
17507672
Genetics. 1982 Dec;102(4):679-90
6764902
Proc Natl Acad Sci U S A. 2002 Jul 23;99(15):9936-41
12105276
Br Med Bull. 1993 Oct;49(4):822-38
8137131
J Mol Biol. 2007 Apr 20;368(1):273-82
17320905
EMBO J. 1995 Sep 1;14(17):4365-73
7556078
Proc Natl Acad Sci U S A. 2008 Feb 19;105(7):2403-8
18268327
Genetics. 1997 Oct;147(2):507-19
9335589
Proc Natl Acad Sci U S A. 2007 Feb 20;104(8):2791-6
17296932
Curr Genet. 2007 May;51(5):309-19
17415568
Proc Natl Acad Sci U S A. 2000 Nov 21;97(24):13045-50
11069287
Science. 1994 Apr 22;264(5158):566-9
7909170
J Virol. 1994 Aug;68(8):4873-8
7913509
Genetics. 1999 Sep;153(1):81-94
10471702
Genetics. 1994 Jul;137(3):659-70
8088511
FEBS Lett. 2007 Jul 31;581(19):3695-701
17509571
Nat Cell Biol. 2005 Nov;7(11):1039-44
16385730
J Mol Biol. 2001 Apr 13;307(5):1247-60
11292339
Mol Cell Biol. 2001 Oct;21(20):7035-46
11564886
Q Rev Biophys. 2006 Feb;39(1):1-55
16772049
Nature. 2005 Jun 9;435(7043):844-8
15944710
Proc Natl Acad Sci U S A. 2002 May 28;99(11):7402-7
12032295
Proc Natl Acad Sci U S A. 1997 Jun 24;94(13):6618-22
9192614
Nature. 2000 Sep 28;407(6803):477-83
11028992
Biochemistry. 2005 Jan 11;44(1):321-8
15628874
Cell. 2001 Jul 27;106(2):171-82
11511345
Nature. 1991 Jul 25;352(6333):340-2
1677164
EMBO J. 2007 Aug 22;26(16):3794-803
17673909
Proc Natl Acad Sci U S A. 2006 Sep 5;103(36):13445-50
16938837
Mol Cell Biol. 2000 Dec;20(23):8916-22
11073991
Nature. 2004 Mar 18;428(6980):323-8
15029196
EMBO J. 2003 May 1;22(9):2071-81
12727874
Curr Biol. 2000 Nov 16;10(22):1443-6
11102806
Science. 1995 Oct 6;270(5233):93-5
7569955
Proc Natl Acad Sci U S A. 2005 Sep 6;102(36):12825-30
16123127
Genetics. 2005 Sep;171(1):23-34
15956663
Proc Natl Acad Sci U S A. 1997 Sep 16;94(19):10012-4
9294153
Biochemistry. 2007 Nov 27;46(47):13505-22
17979302
Proc Natl Acad Sci U S A. 2005 Jul 26;102(30):10575-80
16024723
Nature. 2007 Sep 13;449(7159):233-7
17767153
Cell. 1993 Jul 2;73(7):1339-47
8100741
Mol Cell. 2000 Jan;5(1):163-72
10678178
Mol Cell Biol. 2004 Aug;24(16):7206-13
15282319
J Biol Chem. 2002 Jun 28;277(26):23702-8
11923285
J Biomol NMR. 2006 Feb;34(2):75-87
16518695
Proc Natl Acad Sci U S A. 2002 Dec 10;99 Suppl 4:16384-91
12177423
Nat Biotechnol. 2003 Sep;21(9):1075-81
12910243
Nat Struct Biol. 1995 Nov;2(11):990-8
7583673
Nat Genet. 2008 Apr;40(4):460-5
18362884
EMBO J. 1996 Jun 17;15(12):3127-34
8670813
Mol Microbiol. 2004 Mar;51(6):1649-59
15009892
Mol Cell Biol. 2002 Aug;22(15):5593-605
12101251
Genetics. 2000 Oct;156(2):559-70
11014806
Science. 1999 Feb 26;283(5406):1339-43
10037606
J Bacteriol. 1971 May;106(2):519-22
5573734
J Bacteriol. 1987 Jun;169(6):2598-600
3294799
PLoS One. 2008 May 14;3(5):e2174
18478094
Proc Natl Acad Sci U S A. 2007 Jun 5;104(23):9741-6
17535913
Genes Dev. 2003 Sep 1;17(17):2083-7
12923060
J Biol Chem. 2007 Feb 2;282(5):3004-13
17142456
Science. 1995 May 12;268(5212):880-4
7754373
Microbiol Mol Biol Rev. 2000 Sep;64(3):489-502
10974123
Science. 2004 Jul 30;305(5684):673-6
15286374
Proc Natl Acad Sci U S A. 1997 Sep 2;94(18):9773-8
9275200
J Biol Chem. 1991 May 5;266(13):7963-6
2022624
J Biol Chem. 2003 Oct 31;278(44):43717-27
12917441
J Comp Pathol. 1968 Jul;78(3):293-9
4970191