title: Ubiquitylation and the Fanconi anemia pathway.
abstract: The Fanconi anemia (FA) pathway maintains genome stability through co-ordination of DNA repair of interstrand crosslinks (ICLs). Disruption of the FA pathway yields hypersensitivity to interstrand crosslinking agents, bone marrow failure and cancer predisposition. Early steps in DNA damage dependent activation of the pathway are governed by monoubiquitylation of FANCD2 and FANCI by the intrinsic FA E3 ubiquitin ligase, FANCL. Downstream FA pathway components and associated factors such as FAN1 and SLX4 exhibit ubiquitin-binding motifs that are important for their DNA repair function, underscoring the importance of ubiquitylation in FA pathway mediated repair. Importantly, ubiquitylation provides the foundations for cross-talk between repair pathways, which in concert with the FA pathway, resolve interstrand crosslink damage and maintain genomic stability.
authors: Laboratory of Genome Maintenance, The Rockefeller University, New York, NY 10065, USA.
authors: Elizabeth
authors: E
authors: Garner
authors: Agata
authors: A
authors: Smogorzewska
references: Mol Cell. 2007 Dec 14;28(5):798-809
references: 18082605
references: Mol Cell. 2006 Aug;23(4):589-96
references: 16916645
references: Mol Cell. 2005 Feb 4;17(3):331-9
references: 15694335
references: Cell. 2010 Jul 9;142(1):77-88
references: 20603016
references: Mol Cell. 2008 Oct 10;32(1):118-28
references: 18851838
references: Mol Cell Biol. 2010 Feb;30(4):1088-96
references: 19995904
references: Nat Genet. 2005 Sep;37(9):958-63
references: 16116422
references: Science. 2009 Dec 18;326(5960):1698-701
references: 19965384
references: Mol Gen Genet. 1981;181(3):346-51
references: 7017347
references: Mol Cell. 2006 Oct 6;24(1):115-25
references: 17018297
references: EMBO J. 2007 Jul 11;26(13):3238-49
references: 17581638
references: Cell Oncol. 2007;29(3):211-8
references: 17452773
references: Mol Cell. 2009 Sep 11;35(5):716-23
references: 19748364
references: Nat Genet. 2007 Feb;39(2):159-61
references: 17200672
references: Science. 2007 May 25;316(5828):1160-6
references: 17525332
references: Nat Genet. 2010 May;42(5):406-9
references: 20400963
references: Trends Biochem Sci. 2000 Mar;25(3):112-4
references: 10694879
references: Mol Cell. 2004 Dec 22;16(6):1027-34
references: 15610744
references: Cell. 2009 Jul 10;138(1):63-77
references: 19596235
references: Nature. 2005 Jul 21;436(7049):428-33
references: 15931174
references: Mol Cell Biol. 2004 Dec;24(24):10733-41
references: 15572677
references: Dev Cell. 2009 Feb;16(2):314-20
references: 19217432
references: Mutat Res. 1993 Jan;293(2):99-118
references: 7678147
references: DNA Repair (Amst). 2009 Apr 5;8(4):544-56
references: 19213613
references: Blood. 2008 May 15;111(10):5215-22
references: 18174376
references: Mol Cell. 2008 Nov 7;32(3):313-24
references: 18995830
references: Mol Cell. 2007 Dec 14;28(5):786-97
references: 18082604
references: DNA Repair (Amst). 2006 Nov 8;5(11):1398-406
references: 16914393
references: Mol Cell. 2001 Feb;7(2):249-62
references: 11239454
references: J Biol Chem. 2009 Aug 28;284(35):23182-6
references: 19589784
references: J Biol Chem. 2004 Oct 29;279(44):46035-45
references: 15299017
references: Science. 2003 Jun 6;300(5625):1542-8
references: 12791985
references: Cancer Cell. 2005 Sep;8(3):255-65
references: 16153896
references: DNA Repair (Amst). 2009 Apr 5;8(4):430-5
references: 19264559
references: Nat Rev Genet. 2007 Oct;8(10):735-48
references: 17768402
references: DNA Repair (Amst). 2008 Feb 1;7(2):230-8
references: 18006388
references: Nat Genet. 2011 Feb;43(2):142-6
references: 21240275
references: Mol Cell Biol. 2000 Nov;20(21):7980-90
references: 11027268
references: Mol Cell. 2006 Jul 21;23(2):265-71
references: 16857592
references: Nature. 2002 Sep 12;419(6903):135-41
references: 12226657
references: EMBO J. 2006 Oct 18;25(20):4921-32
references: 17036055
references: DNA Repair (Amst). 2006 Nov 8;5(11):1317-26
references: 16860002
references: Mol Cell Biol. 2003 Jan;23(2):754-61
references: 12509472
references: Cancer Res. 2007 Aug 1;67(15):7395-405
references: 17671210
references: Mol Cell Biol. 2001 Feb;21(3):713-20
references: 11154259
references: Cell. 2009 Jul 10;138(1):78-89
references: 19596236
references: Nat Genet. 2005 Sep;37(9):953-7
references: 16116421
references: Mol Cell. 2008 Dec 26;32(6):767-77
references: 19111657
references: Nat Struct Mol Biol. 2007 Jun;14(6):564-7
references: 17460694
references: Nat Genet. 2003 Oct;35(2):165-70
references: 12973351
references: Nat Struct Mol Biol. 2008 Nov;15(11):1138-46
references: 18931676
references: Proc Natl Acad Sci U S A. 1998 Oct 27;95(22):13085-90
references: 9789045
references: Genetics. 2000 Jul;155(3):1069-81
references: 10880470
references: J Exp Bot. 2011 Mar;62(5):1565-79
references: 21081662
references: EMBO J. 2003 Jun 16;22(12):3188-97
references: 12805232
references: Environ Mol Mutagen. 2010 Jul;51(6):552-66
references: 20658647
references: Mol Cell. 2003 Jul;12(1):247-54
references: 12887909
references: Mol Cell. 2005 Sep 16;19(6):841-7
references: 16168378
references: Genetics. 1980 Jun;95(2):273-88
references: 7009316
references: Nat Genet. 2011 Feb;43(2):147-52
references: 21240276
references: PLoS One. 2010 Oct 13;5(10):e13313
references: 20967207
references: Mol Cell. 2010 Jul 9;39(1):36-47
references: 20603073
references: Cell. 2010 Jul 9;142(1):65-76
references: 20603015
references: Nat Genet. 2005 Sep;37(9):931-3
references: 16116424
references: Nat Genet. 2007 Feb;39(2):162-4
references: 17200671
references: Nat Genet. 2011 Feb;43(2):138-41
references: 21240277
references: Cancer Res. 2005 Dec 15;65(24):11704-11
references: 16357182
references: Mol Cell Biol. 2007 Sep;27(18):6433-45
references: 17636031
references: EMBO Rep. 2004 Oct;5(10):989-93
references: 15359272
references: Science. 2010 Aug 6;329(5992):693-6
references: 20671156
references: Mol Cell. 2002 Sep;10(3):683-91
references: 12408834
references: Mol Cell. 2009 Jul 10;35(1):116-27
references: 19595721
references: Genes Dev. 2009 Mar 1;23(5):555-60
references: 19270156
references: Mol Cell. 2007 Feb 9;25(3):331-43
references: 17289582
references: J Biol Chem. 2009 Oct 9;284(41):27908-27917
references: 19684342
references: Mol Cell. 2004 May 21;14(4):491-500
references: 15149598
references: Science. 2002 Jul 26;297(5581):606-9
references: 12065746
references: Blood. 2009 Mar 5;113(10):2181-90
references: 19109555
references: J Biol Chem. 2005 Jun 24;280(25):23593-8
references: 15849361
references: Mutat Res. 2009 Jul 31;668(1-2):4-10
references: 19622403
references: Environ Mol Mutagen. 2010 Jul;51(6):635-45
references: 20175117
references: Science. 2001 Nov 23;294(5547):1713-6
references: 11721054
references: EMBO J. 2003 Apr 1;22(7):1654-64
references: 12660171
references: Mol Cell. 2004 Aug 27;15(4):607-20
references: 15327776
references: Mol Cell Biol. 2006 Sep;26(18):7005-15
references: 16943440
references: J Biol Chem. 2000 Aug 25;275(34):26632-6
references: 10882712
references: J Biol Chem. 2009 Sep 4;284(36):24443-52
references: 19561358
references: Blood. 2011 May 12;117(19):5078-87
references: 21355096
references: Mol Cell Biol. 2007 Apr;27(8):3098-108
references: 17296736
references: J Cell Biol. 2010 Oct 18;191(2):249-57
references: 20937699
references: Blood. 2009 Jul 2;114(1):174-80
references: 19423727
references: Mol Cell. 2010 Mar 26;37(6):879-86
references: 20347429
references: Mol Cell Biol. 2010 Mar;30(5):1217-30
references: 20028736
references: J Biol Chem. 2010 Apr 2;285(14):10362-9
references: 20147293
references: Proc Natl Acad Sci U S A. 2011 Apr 19;108(16):6492-6
references: 21464321
references: Proc Natl Acad Sci U S A. 1980 Mar;77(3):1365-8
references: 6769112
references: Cell. 2008 Sep 19;134(6):969-80
references: 18805090
references: Cell. 2002 May 17;109(4):459-72
references: 12086603
references: PLoS Genet. 2009 Sep;5(9):e1000645
references: 19763158
references: Nat Genet. 2005 Sep;37(9):934-5
references: 16116423
references: Chembiochem. 2005 Jan;6(1):27-32
references: 15637664
references: J Biol Chem. 2010 Oct 8;285(41):31525-36
references: 20675655
references: Nat Struct Mol Biol. 2010 Mar;17(3):294-8
references: 20154706
references: Cell. 2007 Apr 20;129(2):289-301
references: 17412408
references: Mol Cell Biol. 2004 Oct;24(19):8576-85
references: 15367677
references: Genetics. 2001 Jan;157(1):103-18
references: 11139495