title: Base excision repair.
abstract: Base excision repair (BER) corrects DNA damage from oxidation, deamination and alkylation. Such base lesions cause little distortion to the DNA helix structure. BER is initiated by a DNA glycosylase that recognizes and removes the damaged base, leaving an abasic site that is further processed by short-patch repair or long-patch repair that largely uses different proteins to complete BER. At least 11 distinct mammalian DNA glycosylases are known, each recognizing a few related lesions, frequently with some overlap in specificities. Impressively, the damaged bases are rapidly identified in a vast excess of normal bases, without a supply of energy. BER protects against cancer, aging, and neurodegeneration and takes place both in nuclei and mitochondria. More recently, an important role of uracil-DNA glycosylase UNG2 in adaptive immunity was revealed. Furthermore, other DNA glycosylases may have important roles in epigenetics, thus expanding the repertoire of BER proteins.
authors.affiliation: Department of Cancer Research and Molecular Medicine, Norwegian University of Science and Technology, N-7489 Trondheim, Norway. hans.krokan@ntnu.no
authors.forename: Hans E
authors.initials: HE
authors.lastname: Krokan
authors.forename: Magnar
authors.initials: M
authors.lastname: Bjørås
mesh_terms: D000818:Animals; D002467:Cell Nucleus / Q000378:metabolism; D004247:DNA / Q000235:genetics*; D004249:DNA Damage; D045647:DNA Glycosylases / Q000235:genetics; D019951:DNA Polymerase beta / Q000378:metabolism; D004260:DNA Repair; D044127:Epigenesis, Genetic; D004926:Escherichia coli / Q000201:enzymology; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D008928:Mitochondria / Q000378:metabolism; D008958:Models, Molecular; D016296:Mutagenesis; D009369:Neoplasms / Q000201:enzymology; D010100:Oxygen / Q000737:chemistry; D011065:Poly(ADP-ribose) Polymerases / Q000378:metabolism; D011487:Protein Conformation
chemical_list: D004247:DNA; D011065:Poly(ADP-ribose) Polymerases; D019951:DNA Polymerase beta; C492570:CCNO protein, human; D045647:DNA Glycosylases; D010100:Oxygen
references.citation: DNA Repair (Amst). 2007 Apr 1;6(4):505-16
references.pmid: 17116429
references.citation: Mol Cell Biol. 2008 Aug;28(16):4975-87
references.pmid: 18541666
references.citation: Biochem Pharmacol. 2012 Jul 15;84(2):137-46
references.pmid: 22469522
references.citation: Cell Cycle. 2004 Aug;3(8):998-1001
references.pmid: 15280658
references.citation: Hum Mol Genet. 2002 Nov 1;11(23):2961-7
references.pmid: 12393807
references.citation: Nucleic Acids Res. 2011 Oct;39(19):8430-44
references.pmid: 21745813
references.citation: Mol Cell Biol. 2007 Aug;27(15):5587-96
references.pmid: 17526740
references.citation: Proc Natl Acad Sci U S A. 2009 Apr 7;106(14):5593-8
references.pmid: 19307563
references.citation: Nature. 2011 Feb 17;470(7334):419-23
references.pmid: 21278727
references.citation: EMBO J. 2007 May 2;26(9):2411-20
references.pmid: 17410210
references.citation: DNA Repair (Amst). 2007 Apr 1;6(4):398-409
references.pmid: 17129767
references.citation: Curr Biol. 1997 Jun 1;7(6):397-407
references.pmid: 9197244
references.citation: Nat Rev Mol Cell Biol. 2012 Jun 20;13(7):411-24
references.pmid: 22713970
references.citation: Proc Natl Acad Sci U S A. 1997 Jul 8;94(14):7429-34
references.pmid: 9207108
references.citation: Cancer Res. 2004 Jul 1;64(13):4411-4
references.pmid: 15231648
references.citation: Cancer Lett. 2012 Dec 31;327(1-2):73-89
references.pmid: 22252118
references.citation: Antioxid Redox Signal. 2011 Jun 15;14(12):2491-507
references.pmid: 20649466
references.citation: Cancer Res. 1992 Sep 1;52(17):4824-7
references.pmid: 1511447
references.citation: Nature. 2011 Mar 10;471(7337):245-8
references.pmid: 21390132
references.citation: Br J Cancer. 2004 Apr 19;90(8):1591-3
references.pmid: 15083190
references.citation: Nucleic Acids Res. 2005 Aug 29;33(15):4849-56
references.pmid: 16129732
references.citation: Carcinogenesis. 2005 Mar;26(3):547-55
references.pmid: 15564287
references.citation: Nat Struct Biol. 2002 Sep;9(9):659-64
references.pmid: 12161745
references.citation: Nature. 2011 Mar 10;471(7337):240-4
references.pmid: 21390131
references.citation: Nat Immunol. 2003 Oct;4(10):1023-8
references.pmid: 12958596
references.citation: BMC Cancer. 2006 Oct 09;6:243
references.pmid: 17029639
references.citation: Nature. 1996 Nov 7;384(6604):87-92
references.pmid: 8900285
references.citation: Neuroscience. 2007 Apr 14;145(4):1201-12
references.pmid: 17101234
references.citation: J Biol Chem. 2002 Nov 1;277(44):42205-13
references.pmid: 12200441
references.citation: Mol Microbiol. 2006 Mar;59(5):1602-9
references.pmid: 16468998
references.citation: DNA Repair (Amst). 2008 Nov 1;7(11):1869-81
references.pmid: 18721906
references.citation: Nucleic Acids Res. 2007;35(13):4313-21
references.pmid: 17576692
references.citation: Proc Natl Acad Sci U S A. 2010 Mar 16;107(11):4925-30
references.pmid: 20185759
references.citation: Nature. 2000 Jan 27;403(6768):451-6
references.pmid: 10667800
references.citation: J Biol Chem. 2006 Nov 24;281(47):35649-55
references.pmid: 17005572
references.citation: Nucleic Acids Res. 2011 Apr;39(8):3166-75
references.pmid: 21183466
references.citation: Mol Biol Evol. 2011 Mar;28(3):1307-17
references.pmid: 21135150
references.citation: Proc Natl Acad Sci U S A. 1974 Sep;71(9):3649-53
references.pmid: 4610583
references.citation: Nucleic Acids Res. 1997 Feb 15;25(4):750-5
references.pmid: 9016624
references.citation: Mol Cell. 2007 Sep 7;27(5):829-41
references.pmid: 17803946
references.citation: J Biol Chem. 1999 Jun 25;274(26):18470-6
references.pmid: 10373455
references.citation: Carcinogenesis. 2009 Jul;30(7):1147-54
references.pmid: 19443904
references.citation: Antioxid Redox Signal. 2009 Mar;11(3):601-20
references.pmid: 18976116
references.citation: DNA Repair (Amst). 2011 Apr 3;10(4):445-51
references.pmid: 21315663
references.citation: J Biol Chem. 2003 Dec 12;278(50):49679-84
references.pmid: 14522990
references.citation: DNA Repair (Amst). 2009 Jul 4;8(7):786-94
references.pmid: 19346169
references.citation: Biochemistry. 2004 Oct 12;43(40):12924-30
references.pmid: 15461465
references.citation: FEBS Lett. 2010 Apr 16;584(8):1553-7
references.pmid: 20214901
references.citation: Cell Mol Life Sci. 2010 Nov;67(21):3573-87
references.pmid: 20714778
references.citation: Prog Nucleic Acid Res Mol Biol. 2001;68:xvii-xxx
references.pmid: 11554316
references.citation: Mol Cell Biol. 2007 Aug;27(15):5597-605
references.pmid: 17548475
references.citation: Proc Natl Acad Sci U S A. 2006 Feb 7;103(6):1864-9
references.pmid: 16446448
references.citation: Mol Aspects Med. 2007 Jun-Aug;28(3-4):276-306
references.pmid: 17590428
references.citation: Nat Genet. 1999 Nov;23(3):266-8
references.pmid: 10545939
references.citation: J Biol Chem. 2005 Sep 9;280(36):31641-7
references.pmid: 16002405
references.citation: Nucleic Acids Res. 2007;35(11):3859-67
references.pmid: 17526518
references.citation: Nucleic Acids Res. 1999 Sep 15;27(18):3712-9
references.pmid: 10471741
references.citation: Environ Mol Mutagen. 2010 Jun;51(5):417-26
references.pmid: 20544882
references.citation: J Biol Chem. 2002 Aug 23;277(34):30417-20
references.pmid: 12097317
references.citation: DNA Repair (Amst). 2008 Mar 1;7(3):431-8
references.pmid: 18182332
references.citation: Cancer Res. 1997 Jun 1;57(11):2151-6
references.pmid: 9187114
references.citation: Nucleic Acids Res. 2004 Oct 12;32(18):5486-98
references.pmid: 15479784
references.citation: Proc Natl Acad Sci U S A. 2000 Dec 5;97(25):13573-8
references.pmid: 11106395
references.citation: Mutat Res. 2005 Sep 4;577(1-2):55-76
references.pmid: 15941573
references.citation: Nucleic Acids Res. 2007;35(12):3879-92
references.pmid: 17537817
references.citation: EMBO J. 2000 Feb 15;19(4):758-66
references.pmid: 10675345
references.citation: Immunol Res. 2010 Jul;47(1-3):113-22
references.pmid: 20082153
references.citation: Proc Natl Acad Sci U S A. 1997 Jul 8;94(14):7303-7
references.pmid: 9207086
references.citation: Mol Cell. 2008 Nov 7;32(3):325-36
references.pmid: 18995831
references.citation: Proc Natl Acad Sci U S A. 1997 Jul 22;94(15):8016-20
references.pmid: 9223306
references.citation: J Immunol. 2007 Nov 1;179(9):6064-71
references.pmid: 17947680
references.citation: J Cell Physiol. 2013 Feb;228(2):258-66
references.pmid: 22718094
references.citation: Cell Res. 2008 Jan;18(1):27-47
references.pmid: 18166975
references.citation: Proc Natl Acad Sci U S A. 2011 Nov 15;108(46):18802-7
references.pmid: 22065741
references.citation: Cell Mol Life Sci. 2009 Mar;66(6):981-93
references.pmid: 19153658
references.citation: Chemistry. 2008;14(3):786-93
references.pmid: 18000994
references.citation: Biochim Biophys Acta. 2010 Jan-Feb;1799(1-2):119-30
references.pmid: 20123074
references.citation: Proc Natl Acad Sci U S A. 1997 Jan 7;94(1):109-14
references.pmid: 8990169
references.citation: DNA Repair (Amst). 2008 Apr 2;7(4):605-16
references.pmid: 18295553
references.citation: Cell. 2011 Apr 15;145(2):198-211
references.pmid: 21496641
references.citation: Environ Mol Mutagen. 2011 Oct;52(8):623-35
references.pmid: 21786338
references.citation: DNA Repair (Amst). 2007 Jun 1;6(6):695-711
references.pmid: 17337257
references.citation: Biochem Pharmacol. 2008 Sep 15;76(6):697-706
references.pmid: 18599024
references.citation: Mol Biol Evol. 2003 Oct;20(10):1603-11
references.pmid: 12832627
references.citation: Nucleic Acids Res. 2002 Nov 15;30(22):4926-36
references.pmid: 12433996
references.citation: EMBO J. 1997 Oct 15;16(20):6314-22
references.pmid: 9321410
references.citation: DNA Repair (Amst). 2002 Jul 17;1(7):517-29
references.pmid: 12509226
references.citation: Biochemistry. 2010 Mar 2;49(8):1658-66
references.pmid: 20099873
references.citation: DNA Repair (Amst). 2007 Aug 1;6(8):1100-15
references.pmid: 17485252
references.citation: FEBS J. 2005 Apr;272(8):2012-21
references.pmid: 15819892
references.citation: Mol Cell Biol. 2003 Nov;23(22):8386-94
references.pmid: 14585995
references.citation: Annu Rev Biochem. 2007;76:1-22
references.pmid: 17328676
references.citation: Nucleic Acids Res. 2007;35(7):2451-9
references.pmid: 17395642
references.citation: DNA Repair (Amst). 2012 Jun 1;11(6):587-93
references.pmid: 22483865
references.citation: DNA Repair (Amst). 2006 Feb 3;5(2):189-209
references.pmid: 16290067
references.citation: Hum Mol Genet. 2012 Nov 15;21(22):4939-47
references.pmid: 22914735
references.citation: Nature. 2007 Jun 21;447(7147):941-50
references.pmid: 17581577
references.citation: Cell. 2011 Jul 8;146(1):67-79
references.pmid: 21722948
references.citation: DNA Repair (Amst). 2008 Nov 1;7(11):1916-23
references.pmid: 18723127
references.citation: EMBO J. 2003 Oct 1;22(19):4898-909
references.pmid: 14517230
references.citation: Cell. 1995 Mar 24;80(6):869-78
references.pmid: 7697717
references.citation: Curr Biol. 2002 Oct 15;12(20):1748-55
references.pmid: 12401169
references.citation: Nature. 2007 May 24;447(7143):447-52
references.pmid: 17450122
references.citation: Chem Rev. 2006 Feb;106(2):361-82
references.pmid: 16464010
references.citation: Biochemistry. 2012 Jul 3;51(26):5269-75
references.pmid: 22650412
references.citation: Genes Dev. 1995 Mar 1;9(5):509-20
references.pmid: 7698643
references.citation: Proc Natl Acad Sci U S A. 2012 Sep 11;109(37):14948-53
references.pmid: 22927410
references.citation: Mol Cell. 2000 Jun;5(6):1059-65
references.pmid: 10912000
references.citation: J Biol Chem. 2004 Apr 2;279(14):13601-6
references.pmid: 14729667
references.citation: Oncogene. 2002 Dec 16;21(58):8935-48
references.pmid: 12483510
references.citation: Proc Natl Acad Sci U S A. 2011 Dec 27;108(52):21105-10
references.pmid: 22160723
references.citation: Nucleic Acids Res. 2012 Jun;40(11):4841-9
references.pmid: 22362737
references.citation: J Mol Biol. 2008 Aug 1;381(1):13-23
references.pmid: 18585735
references.citation: DNA Repair (Amst). 2007 Apr 1;6(4):489-504
references.pmid: 17116428
references.citation: DNA Repair (Amst). 2008 Sep 1;7(9):1578-88
references.pmid: 18603019
references.citation: Biochemistry. 2010 Feb 16;49(6):1053-5
references.pmid: 20067321
references.citation: Proc Natl Acad Sci U S A. 1997 Jul 22;94(15):8010-5
references.pmid: 9223305
references.citation: Prog Nucleic Acid Res Mol Biol. 2001;68:57-74
references.pmid: 11554313
references.citation: DNA Repair (Amst). 2007 Apr 1;6(4):530-43
references.pmid: 17113833
references.citation: J Biol Chem. 2012 Jul 6;287(28):23840-9
references.pmid: 22573322
references.citation: DNA Repair (Amst). 2009 Jul 4;8(7):834-43
references.pmid: 19442590
references.citation: J Exp Med. 2012 Jul 2;209(7):1379-89
references.pmid: 22665573
references.citation: J Biol Chem. 2007 Mar 23;282(12):9182-94
references.pmid: 17114185
references.citation: Oncogene. 2007 Dec 10;26(56):7780-91
references.pmid: 18066091
references.citation: Nature. 2008 Apr 24;452(7190):961-5
references.pmid: 18432238
references.citation: EMBO J. 1999 Jul 1;18(13):3834-44
references.pmid: 10393198
references.citation: FEMS Microbiol Rev. 2009 Nov;33(6):1044-78
references.pmid: 19659577
references.citation: J Biol Chem. 1999 May 21;274(21):15230-6
references.pmid: 10329732
references.citation: Adv Immunol. 2007;94:37-73
references.pmid: 17560271
references.citation: Proc Natl Acad Sci U S A. 2007 Oct 23;104(43):17010-5
references.pmid: 17940040
references.citation: PLoS One. 2010 Aug 18;5(8):e12229
references.pmid: 20805875
references.citation: Adv Immunol. 2010;105:159-91
references.pmid: 20510733
references.citation: Cell. 1996 Jul 26;86(2):321-9
references.pmid: 8706136
references.citation: Gastroenterology. 2007 Dec;133(6):1858-68
references.pmid: 18054558
references.citation: Chromosoma. 2012 Feb;121(1):1-20
references.pmid: 22048164
references.citation: EMBO J. 2008 Jan 9;27(1):51-61
references.pmid: 18079698
references.citation: J Exp Med. 2007 Nov 26;204(12):3017-26
references.pmid: 18025127
references.citation: Mol Cell Biol. 2007 Sep;27(17):5949-56
references.pmid: 17591702
references.citation: Arch Biochem Biophys. 2001 Dec 1;396(1):1-9
references.pmid: 11716455
references.citation: Science. 1994 Jul 1;265(5168):103-6
references.pmid: 8016642
references.citation: DNA Repair (Amst). 2005 Jan 2;4(1):41-50
references.pmid: 15533836
references.citation: FEBS Lett. 2012 Jun 21;586(13):1821-3
references.pmid: 22641038
references.citation: J Biol Chem. 2004 Jun 25;279(26):26876-84
references.pmid: 15126496
references.citation: J Biol Chem. 2003 Nov 28;278(48):48012-20
references.pmid: 13129925
references.citation: Immunol Rev. 2004 Aug;200:156-64
references.pmid: 15242403
references.citation: Chem Rev. 2006 Feb;106(2):506-55
references.pmid: 16464017
references.citation: Mol Cell Biol. 2007 Oct;27(20):7198-205
references.pmid: 17698580
references.citation: Cell. 1998 Oct 16;95(2):249-58
references.pmid: 9790531
references.citation: Proc Natl Acad Sci U S A. 2002 Mar 19;99(6):3523-8
references.pmid: 11904416
references.citation: J Cereb Blood Flow Metab. 2011 Feb;31(2):680-92
references.pmid: 20736962
references.citation: Cancer Res. 2004 May 1;64(9):3096-102
references.pmid: 15126346
references.citation: DNA Repair (Amst). 2012 Jun 1;11(6):559-69
references.pmid: 22521144
references.citation: EMBO J. 2000 Aug 1;19(15):3857-69
references.pmid: 10921868
references.citation: J Clin Invest. 2008 Jul;118(7):2516-25
references.pmid: 18521188
references.citation: Prog Neurobiol. 2011 Jul;94(2):166-200
references.pmid: 21550379
references.citation: J Clin Invest. 2004 Jun;113(12):1711-21
references.pmid: 15199406
references.citation: J Biol Chem. 2008 Sep 26;283(39):26349-56
references.pmid: 18635552
references.citation: Methods Enzymol. 2006;408:91-107
references.pmid: 16793365
references.citation: Trends Immunol. 2011 May;32(5):194-201
references.pmid: 21493144
references.citation: Nucleic Acids Res. 2012 Jul;40(13):6016-25
references.pmid: 22447450
references.citation: Nucleic Acids Res. 2012 Sep 1;40(17):8276-84
references.pmid: 22740654
references.citation: J Biol Chem. 2008 Oct 3;283(40):27028-37
references.pmid: 18662981
references.citation: Biochemistry. 2007 Apr 3;46(13):4158-63
references.pmid: 17348689
references.citation: DNA Repair (Amst). 2012 Mar 1;11(3):294-303
references.pmid: 22226374
references.citation: DNA Repair (Amst). 2008 Jun 1;7(6):932-40
references.pmid: 18472309
references.citation: Nucleic Acids Res. 1998 Nov 15;26(22):5123-33
references.pmid: 9801309
references.citation: Cell Mol Life Sci. 2010 Nov;67(21):3633-47
references.pmid: 20844920
references.citation: Trends Neurosci. 2004 Oct;27(10):595-600
references.pmid: 15374670
references.citation: J Clin Invest. 2012 Jul;122(7):2680-9
references.pmid: 22684101
references.citation: Nature. 1993 Apr 22;362(6422):709-15
references.pmid: 8469282
references.citation: Nat Struct Mol Biol. 2004 Dec;11(12):1230-6
references.pmid: 15558051
references.citation: Biochemistry. 2010 Jun 22;49(24):4957-67
references.pmid: 20469926