FANCD2

Summary

Gene Symbol: FANCD2
Description: Fanconi anemia complementation group D2
Alias: FA-D2, FA4, FACD, FAD, FAD2, FANCD, Fanconi anemia group D2 protein
Species: human
Products:     FANCD2

Top Publications

  1. Park H, Wang H, Zhang J, Datta S, Fei P. Convergence of Rad6/Rad18 and Fanconi anemia tumor suppressor pathways upon DNA damage. PLoS ONE. 2010;5:e13313 pubmed publisher
    ..We previously reported that FANCD2 monoubiquitination, a hallmark of the FA pathway activation, can be regulated by HHR6...
  2. Murina O, von Aesch C, Karakus U, Ferretti L, Bolck H, Hänggi K, et al. FANCD2 and CtIP cooperate to repair DNA interstrand crosslinks. Cell Rep. 2014;7:1030-8 pubmed publisher
    ..b>FANCD2 monoubiquitination and CtIP-dependent DNA-end resection represent key events in FA and HR activation, respectively,..
  3. Song I, Barkley L, Day T, Weiss R, Vaziri C. A novel role for Fanconi anemia (FA) pathway effector protein FANCD2 in cell cycle progression of untransformed primary human cells. Cell Cycle. 2010;9:2375-88 pubmed
    ..The Fanconia Anemia D2 (FANCD2) protein is implicated in repair of various forms of DNA damage including ICLs...
  4. Ishiai M, Kitao H, Smogorzewska A, Tomida J, Kinomura A, Uchida E, et al. FANCI phosphorylation functions as a molecular switch to turn on the Fanconi anemia pathway. Nat Struct Mol Biol. 2008;15:1138-46 pubmed publisher
    ..DNA damage or replication fork stress, the Fanconi anemia pathway is activated, leading to monoubiquitination of FANCD2 and FANCI and their colocalization in foci...
  5. Bogliolo M, Lyakhovich A, Callen E, Castella M, Cappelli E, Ramirez M, et al. Histone H2AX and Fanconi anemia FANCD2 function in the same pathway to maintain chromosome stability. EMBO J. 2007;26:1340-51 pubmed
    ..The central FA protein FANCD2 is known to relocate to chromatin upon DNA damage in a poorly understood process...
  6. Barroso E, Milne R, Fernandez L, Zamora P, Arias J, Benitez J, et al. FANCD2 associated with sporadic breast cancer risk. Carcinogenesis. 2006;27:1930-7 pubmed
    ..We studied seven SNPs in FANCA, FANCL and FANCD2 in a total of 897 consecutive and non-related sporadic breast cancer cases and 1033 unaffected controls from the ..
  7. Gordon S, Alon N, Buchwald M. FANCC, FANCE, and FANCD2 form a ternary complex essential to the integrity of the Fanconi anemia DNA damage response pathway. J Biol Chem. 2005;280:36118-25 pubmed
    ..of seven of the nine identified FA genes participate in a protein complex required for monoubiquitination of the FANCD2 protein...
  8. Park W, Margossian S, Horwitz A, Simons A, D Andrea A, Parvin J. Direct DNA binding activity of the Fanconi anemia D2 protein. J Biol Chem. 2005;280:23593-8 pubmed
    ..In these experiments we purified full-length Fanconi anemia protein D2 (FANCD2), and we found that FANCD2 bound to DNA with specificity for certain structures: double strand DNA ends and ..
  9. Yan Z, Guo R, Paramasivam M, Shen W, Ling C, Fox D, et al. A ubiquitin-binding protein, FAAP20, links RNF8-mediated ubiquitination to the Fanconi anemia DNA repair network. Mol Cell. 2012;47:61-75 pubmed publisher
    ..Both RNF8 and FAAP20 are required for recruitment of FA core complex and FANCD2 to ICLs, whereas RNF168 can modulate efficiency of the recruitment...

More Information

Publications63

  1. Sato K, Ishiai M, Toda K, Furukoshi S, Osakabe A, Tachiwana H, et al. Histone chaperone activity of Fanconi anemia proteins, FANCD2 and FANCI, is required for DNA crosslink repair. EMBO J. 2012;31:3524-36 pubmed publisher
    ..A key FA protein, FANCD2, is targeted to chromatin with its partner, FANCI, and plays a critical role in DNA crosslink repair...
  2. Karanja K, Cox S, Duxin J, Stewart S, Campbell J. DNA2 and EXO1 in replication-coupled, homology-directed repair and in the interplay between HDR and the FA/BRCA network. Cell Cycle. 2012;11:3983-96 pubmed publisher
    ..to function in replication-coupled repair of ICLs in conjunction with the Fanconi anemia (FA) regulatory factors FANCD2-FANCI, and, conversely, the FA gene products have been shown to play roles in stalled replication fork rescue even ..
  3. Meetei A, de Winter J, Medhurst A, Wallisch M, Waisfisz Q, van de Vrugt H, et al. A novel ubiquitin ligase is deficient in Fanconi anemia. Nat Genet. 2003;35:165-70 pubmed
    ..1,2). A key step in this pathway is monoubiquitination of FANCD2, resulting in the redistribution of FANCD2 to nuclear foci containing BRCA1 (ref. 3)...
  4. Rudland P, Platt Higgins A, Davies L, De Silva Rudland S, Wilson J, Aladwani A, et al. Significance of the Fanconi anemia FANCD2 protein in sporadic and metastatic human breast cancer. Am J Pathol. 2010;176:2935-47 pubmed publisher
    b>FANCD2, a pivotal protein in the Fanconi anemia and BRCA pathway/network, is monoubiquitylated in the nucleus in response to DNA damage...
  5. Zhi G, Wilson J, Chen X, Krause D, Xiao Y, Jones N, et al. Fanconi anemia complementation group FANCD2 protein serine 331 phosphorylation is important for fanconi anemia pathway function and BRCA2 interaction. Cancer Res. 2009;69:8775-83 pubmed publisher
    ..failure and cancer susceptibility syndrome with at least 13 complementation groups (FANCA, FANCB, FANCC, FANCD1, FANCD2, FANCE, FANCF, FANCG, FANCI, FANCJ, FANCL, FANCM, and FANCN)...
  6. Shen X, Do H, Li Y, Chung W, Tomasz M, de Winter J, et al. Recruitment of fanconi anemia and breast cancer proteins to DNA damage sites is differentially governed by replication. Mol Cell. 2009;35:716-23 pubmed publisher
    ..b>FANCD2, but not FANCJ and FANCN, requires the FA core complex for its recruitment...
  7. Liu T, Ghosal G, Yuan J, Chen J, Huang J. FAN1 acts with FANCI-FANCD2 to promote DNA interstrand cross-link repair. Science. 2010;329:693-6 pubmed publisher
    ..A central event in the FA pathway is mono-ubiquitylation of the FANCI-FANCD2 (ID) protein complex...
  8. Wilson J, Johnson M, Stuckert A, Trueman K, May S, Bryant P, et al. The Chinese hamster FANCG/XRCC9 mutant NM3 fails to express the monoubiquitinated form of the FANCD2 protein, is hypersensitive to a range of DNA damaging agents and exhibits a normal level of spontaneous sister chromatid exchange. Carcinogenesis. 2001;22:1939-46 pubmed
    ..containing the FANCA, FANCC, FANCE, FANCF and FANCG proteins is needed for the activation of a sixth FA protein FANCD2. When monoubiquitinated, the FANCD2 protein co-localizes with the breast cancer susceptibility protein BRCA1 in ..
  9. Taniguchi T, Garcia Higuera I, Xu B, Andreassen P, Gregory R, Kim S, et al. Convergence of the fanconi anemia and ataxia telangiectasia signaling pathways. Cell. 2002;109:459-72 pubmed
    ..Here, we identify the Fanconi anemia protein, FANCD2, as a link between the FA and ATM damage response pathways. ATM phosphorylates FANCD2 on serine 222 in vitro...
  10. Unno J, Itaya A, Taoka M, Sato K, Tomida J, Sakai W, et al. FANCD2 binds CtIP and regulates DNA-end resection during DNA interstrand crosslink repair. Cell Rep. 2014;7:1039-47 pubmed publisher
    ..A key FA protein, FANCD2, is monoubiquitinated and accumulates in chromatin in response to DNA interstrand crosslinks (ICLs), where it ..
  11. Gurtan A, Stuckert P, D Andrea A. The WD40 repeats of FANCL are required for Fanconi anemia core complex assembly. J Biol Chem. 2006;281:10896-905 pubmed
    ..form a multisubunit nuclear complex (FA core complex) required for monoubiquitination of a downstream FA protein, FANCD2. FANCL, which possesses three WD40 repeats and a plant homeodomain (PHD), is the putative E3 ubiquitin ligase ..
  12. Nakanishi K, Yang Y, Pierce A, Taniguchi T, Digweed M, D Andrea A, et al. Human Fanconi anemia monoubiquitination pathway promotes homologous DNA repair. Proc Natl Acad Sci U S A. 2005;102:1110-5 pubmed
    ..DNA-damaging agents activate the monoubiquitination of FANCD2, resulting in its targeting to nuclear foci that also contain BRCA1 and BRCA2/FANCD1, proteins involved in ..
  13. Rego M, Kolling F, Vuono E, Mauro M, Howlett N. Regulation of the Fanconi anemia pathway by a CUE ubiquitin-binding domain in the FANCD2 protein. Blood. 2012;120:2109-17 pubmed publisher
    ..A key step in FA-BRCA pathway activation is the covalent attachment of monoubiquitin to FANCD2 and FANCI...
  14. Wang X, Andreassen P, D Andrea A. Functional interaction of monoubiquitinated FANCD2 and BRCA2/FANCD1 in chromatin. Mol Cell Biol. 2004;24:5850-62 pubmed
    ..Here we show that monoubiquitination of FANCD2, which is activated by DNA damage, is required for targeting of FANCD2 to chromatin, where it interacts with BRCA2...
  15. Sato K, Toda K, Ishiai M, Takata M, Kurumizaka H. DNA robustly stimulates FANCD2 monoubiquitylation in the complex with FANCI. Nucleic Acids Res. 2012;40:4553-61 pubmed publisher
    FANCI and FANCD2 form a complex, and play essential roles in the repair of interstrand DNA crosslinks (ICLs) by the Fanconi anemia (FA) pathway...
  16. Shigechi T, Tomida J, Sato K, Kobayashi M, Eykelenboom J, Pessina F, et al. ATR-ATRIP kinase complex triggers activation of the Fanconi anemia DNA repair pathway. Cancer Res. 2012;72:1149-56 pubmed publisher
    ..of the Fanconi anemia (FA) protein FANCI, triggering its monoubiquitination of the key DNA repair factor FANCD2 by the FA core E3 ligase complex, thereby promoting this central pathway of DNA repair which permits replication ..
  17. Fu D, Dudimah F, Zhang J, Pickering A, Paneerselvam J, Palrasu M, et al. Recruitment of DNA polymerase eta by FANCD2 in the early response to DNA damage. Cell Cycle. 2013;12:803-9 pubmed publisher
    How Fanconi anemia (FA) protein D2 (FANCD2) performs DNA damage repair remains largely elusive. We report here that translesion synthesis DNA polymerase (pol) eta is a novel mediator of FANCD2 function...
  18. Abraham J, Balbo S, Crabb D, Brooks P. Alcohol metabolism in human cells causes DNA damage and activates the Fanconi anemia-breast cancer susceptibility (FA-BRCA) DNA damage response network. Alcohol Clin Exp Res. 2011;35:2113-20 pubmed publisher
    ..resulted in the activation of the FA-BRCA DNA damage response network, as indicated by a monoubiquitination of FANCD2 and phosphorylation of BRCA1. Ser 1524 was identified as 1 site of BRCA1 phosphorylation...
  19. Gordon S, Buchwald M. Fanconi anemia protein complex: mapping protein interactions in the yeast 2- and 3-hybrid systems. Blood. 2003;102:136-41 pubmed
    ..The FANCA, FANCC, FANCE, FANCF, and FANCG proteins form a nuclear complex required for the monoubiquination of the FANCD2 protein...
  20. Wang J, Sarkar T, Zhou M, Sharan S, Ritt D, Veenstra T, et al. CCAAT/enhancer binding protein delta (C/EBPdelta, CEBPD)-mediated nuclear import of FANCD2 by IPO4 augments cellular response to DNA damage. Proc Natl Acad Sci U S A. 2010;107:16131-6 pubmed publisher
    ..we found that C/EBPδ promotes monoubiquitination of the Fanconi anemia complementation group D2 protein (FANCD2), which is necessary for its function in replication-associated DNA repair...
  21. Lyakhovich A, Ramirez M, Castellanos A, Castella M, Simons A, Parvin J, et al. Fanconi anemia protein FANCD2 inhibits TRF1 polyADP-ribosylation through tankyrase1-dependent manner. Genome Integr. 2011;2:4 pubmed publisher
    ..The key FA protein FANCD2 crosstalks with members of DNA damage and repair pathways that also play a role at telomeres...
  22. Kratz K, Schöpf B, Kaden S, Sendoel A, Eberhard R, Lademann C, et al. Deficiency of FANCD2-associated nuclease KIAA1018/FAN1 sensitizes cells to interstrand crosslinking agents. Cell. 2010;142:77-88 pubmed publisher
    ..The link of KIAA1018 to the FA pathway is further strengthened by its recruitment to DNA damage through interaction of its UBZ domain with monoubiquitylated FANCD2. We therefore propose to name KIAA1018 FANCD2-associated nuclease, FAN1.
  23. MacKay C, Déclais A, Lundin C, Agostinho A, Deans A, MacArtney T, et al. Identification of KIAA1018/FAN1, a DNA repair nuclease recruited to DNA damage by monoubiquitinated FANCD2. Cell. 2010;142:65-76 pubmed publisher
    ..The FA core complex catalyzes the monoubiquitination of FANCD2, and this event is essential for several steps of ICL repair...
  24. Pace P, Mosedale G, Hodskinson M, Rosado I, Sivasubramaniam M, Patel K. Ku70 corrupts DNA repair in the absence of the Fanconi anemia pathway. Science. 2010;329:219-23 pubmed publisher
    ..Ku70 binds directly to free DNA ends, committing them to NHEJ repair. We show that purified FANCD2, a downstream effector of the FA pathway, might antagonize Ku70 activity by modifying such DNA substrates...
  25. Longerich S, Kwon Y, Tsai M, Hlaing A, Kupfer G, Sung P. Regulation of FANCD2 and FANCI monoubiquitination by their interaction and by DNA. Nucleic Acids Res. 2014;42:5657-70 pubmed publisher
    b>FANCD2 and FANCI function together in the Fanconi anemia network of deoxyribonucleic acid (DNA) crosslink repair...
  26. Williams S, Longerich S, Sung P, Vaziri C, Kupfer G. The E3 ubiquitin ligase RAD18 regulates ubiquitylation and chromatin loading of FANCD2 and FANCI. Blood. 2011;117:5078-87 pubmed publisher
    ..this pathway is the monoubiquitylation and chromatin localization of 2 FA proteins, FA complementation group D2 (FANCD2) and FANCI...
  27. Garcia Higuera I, Taniguchi T, Ganesan S, Meyn M, Timmers C, Hejna J, et al. Interaction of the Fanconi anemia proteins and BRCA1 in a common pathway. Mol Cell. 2001;7:249-62 pubmed
    ..a nuclear complex containing the FANCA, FANCC, FANCF, and FANCG proteins is required for the activation of the FANCD2 protein to a monoubiquitinated isoform...
  28. Williams S, Wilson J, Clark A, Mitson Salazar A, Tomashevski A, Ananth S, et al. Functional and physical interaction between the mismatch repair and FA-BRCA pathways. Hum Mol Genet. 2011;20:4395-410 pubmed publisher
    ..Activation of this pathway leads to the monoubiquitylation and chromatin localization of FANCD2 and FANCI. It has previously been shown that FANCJ interacts with the mismatch repair (MMR) complex MutL?...
  29. Moldovan G, Madhavan M, Mirchandani K, McCaffrey R, Vinciguerra P, D Andrea A. DNA polymerase POLN participates in cross-link repair and homologous recombination. Mol Cell Biol. 2010;30:1088-96 pubmed publisher
    ..Our data indicate that this novel polymerase-helicase complex participates in homologous recombination repair and is essential for cellular protection against DNA cross-links. ..
  30. Timmers C, Taniguchi T, Hejna J, Reifsteck C, Lucas L, Bruun D, et al. Positional cloning of a novel Fanconi anemia gene, FANCD2. Mol Cell. 2001;7:241-8 pubmed
    ..Complementation group D is heterogeneous, consisting of two distinct genes, FANCD1 and FANCD2. Here we report the positional cloning of FANCD2...
  31. Taniguchi T, Garcia Higuera I, Andreassen P, Gregory R, Grompe M, D Andrea A. S-phase-specific interaction of the Fanconi anemia protein, FANCD2, with BRCA1 and RAD51. Blood. 2002;100:2414-20 pubmed
    ..DNA damage activates this pathway, leading to monoubiquitination of the downstream FANCD2 protein and targeting to nuclear foci containing BRCA1...
  32. Vandenberg C, Gergely F, Ong C, Pace P, Mallery D, Hiom K, et al. BRCA1-independent ubiquitination of FANCD2. Mol Cell. 2003;12:247-54 pubmed
    Monoubiquitination of the FANCD2 protein is a key step in the Fanconi anemia (FA) tumor suppressor pathway, coinciding with this molecule's accumulation at sites of genome damage...
  33. Hussain S, Wilson J, Medhurst A, Hejna J, Witt E, Ananth S, et al. Direct interaction of FANCD2 with BRCA2 in DNA damage response pathways. Hum Mol Genet. 2004;13:1241-8 pubmed
    ..proteins encoded by the known FA genes form a nuclear complex which is required for the monoubiquitination of the FANCD2 protein...
  34. Meetei A, Levitus M, Xue Y, Medhurst A, Zwaan M, Ling C, et al. X-linked inheritance of Fanconi anemia complementation group B. Nat Genet. 2004;36:1219-24 pubmed
    ..group B is an essential component of the nuclear protein 'core complex' responsible for monoubiquitination of FANCD2, a key event in the DNA-damage response pathway associated with Fanconi anemia and BRCA...
  35. Howlett N, Taniguchi T, Durkin S, D Andrea A, Glover T. The Fanconi anemia pathway is required for the DNA replication stress response and for the regulation of common fragile site stability. Hum Mol Genet. 2005;14:693-701 pubmed
    ..damaging agents, and during S-phase of the cell cycle, the FA pathway is activated via the mono-ubiquitination of FANCD2 (FANCD2-Ub), signaling its translocation to discrete nuclear foci, where it co-localizes with the central DNA ..
  36. Howlett N, Harney J, Rego M, Kolling F, Glover T. Functional interaction between the Fanconi Anemia D2 protein and proliferating cell nuclear antigen (PCNA) via a conserved putative PCNA interaction motif. J Biol Chem. 2009;284:28935-42 pubmed publisher
    ..Activation of this pathway occurs via the mono-ubiquitination of the FANCD2 protein, targeting it to nuclear foci where it co-localizes with FANCD1/BRCA2, RAD51, and PCNA...
  37. Fan Q, Zhang F, Barrett B, Ren K, Andreassen P. A role for monoubiquitinated FANCD2 at telomeres in ALT cells. Nucleic Acids Res. 2009;37:1740-54 pubmed publisher
    ..Because of these similarities, we have investigated whether there is a relationship between the FA protein, FANCD2 and telomeres...
  38. Smogorzewska A, Desetty R, Saito T, Schlabach M, Lach F, Sowa M, et al. A genetic screen identifies FAN1, a Fanconi anemia-associated nuclease necessary for DNA interstrand crosslink repair. Mol Cell. 2010;39:36-47 pubmed publisher
    ..complex in a manner dependent on FAN1's ubiquitin-binding domain (UBZ), the ID complex, and monoubiquitination of FANCD2. FAN1 possesses intrinsic 5'-3' exonuclease activity and endonuclease activity that cleaves nicked and branched ..
  39. Hejna J, Bruun D, Pauw D, Moses R. A FANCD2 domain activates Tip60-dependent apoptosis. Cell Biol Int. 2010;34:893-9 pubmed publisher
    The FA (Fanconi anaemia) FANCD2 protein is pivotal in the cellular response to DNA interstrand cross-links. Establishing cells expressing exogenous FANCD2 has proven to be difficult compared with other DNA repair genes...
  40. Pace P, Johnson M, Tan W, Mosedale G, Sng C, Hoatlin M, et al. FANCE: the link between Fanconi anaemia complex assembly and activity. EMBO J. 2002;21:3414-23 pubmed
    ..It activates the downstream protein FANCD2 by monoubiquitylation; this then forges an association with the BRCA1 protein at sites of DNA damage...
  41. Nakanishi K, Taniguchi T, Ranganathan V, New H, Moreau L, Stotsky M, et al. Interaction of FANCD2 and NBS1 in the DNA damage response. Nat Cell Biol. 2002;4:913-20 pubmed
    ..In wild-type cells, MMC activates the colocalization of the FA subtype D2 protein (FANCD2) and NBS1 protein in subnuclear foci...
  42. Yuan F, El Hokayem J, Zhou W, Zhang Y. FANCI protein binds to DNA and interacts with FANCD2 to recognize branched structures. J Biol Chem. 2009;284:24443-52 pubmed publisher
    ..When co-expressed in insect cells, a small fraction of FANCI forms a stable complex with FANCD2 (Fanconi anemia complementation group D2)...
  43. Hölzel M, van Diest P, Bier P, Wallisch M, Hoatlin M, Joenje H, et al. FANCD2 protein is expressed in proliferating cells of human tissues that are cancer-prone in Fanconi anaemia. J Pathol. 2003;201:198-203 pubmed
    ..At least seven distinct FA proteins function in concert to protect the genome, a key step being the activation of FANCD2 by mono-ubiquitination...
  44. Andreassen P, D Andrea A, Taniguchi T. ATR couples FANCD2 monoubiquitination to the DNA-damage response. Genes Dev. 2004;18:1958-63 pubmed
    ..The FA pathway regulates the monoubiquitination of FANCD2 and the assembly of damage-associated FANCD2 nuclear foci...
  45. Montes De Oca R, Andreassen P, Margossian S, Gregory R, Taniguchi T, Wang X, et al. Regulated interaction of the Fanconi anemia protein, FANCD2, with chromatin. Blood. 2005;105:1003-9 pubmed
    DNA damage activates the monoubiquitination of the Fanconi anemia (FA) protein, FANCD2, resulting in the assembly of FANCD2 nuclear foci...
  46. Sims A, Spiteri E, Sims R, Arita A, Lach F, Landers T, et al. FANCI is a second monoubiquitinated member of the Fanconi anemia pathway. Nat Struct Mol Biol. 2007;14:564-7 pubmed
    Activation of the Fanconi anemia (FA) DNA damage-response pathway results in the monoubiquitination of FANCD2, which is regulated by the nuclear FA core ubiquitin ligase complex...
  47. Spardy N, Duensing A, Hoskins E, Wells S, Duensing S. HPV-16 E7 reveals a link between DNA replication stress, fanconi anemia D2 protein, and alternative lengthening of telomere-associated promyelocytic leukemia bodies. Cancer Res. 2008;68:9954-63 pubmed publisher
    ..proteins that are involved in the response to DNA replication stress, most notably the Fanconi anemia D2 protein (FANCD2) as well as BRCA2 and MUS81...
  48. Meetei A, Medhurst A, Ling C, Xue Y, Singh T, Bier P, et al. A human ortholog of archaeal DNA repair protein Hef is defective in Fanconi anemia complementation group M. Nat Genet. 2005;37:958-63 pubmed
    ..Each protein in this complex is essential for monoubiquitination of FANCD2, a key reaction in the Fanconi anemia DNA damage-response pathway...
  49. Hodson C, Cole A, Lewis L, Miles J, Purkiss A, Walden H. Structural analysis of human FANCL, the E3 ligase in the Fanconi anemia pathway. J Biol Chem. 2011;286:32628-37 pubmed publisher
    ..At the heart of this pathway is the monoubiquitination of the FANCI-FANCD2 (ID) complex by the multiprotein "core complex" containing the E3 ubiquitin ligase FANCL...
  50. Long D, R schle M, Joukov V, Walter J. Mechanism of RAD51-dependent DNA interstrand cross-link repair. Science. 2011;333:84-7 pubmed publisher
    ..Recombination acts downstream of FANCI-FANCD2, yet RAD51 binds ICL-stalled replication forks independently of FANCI-FANCD2 and before DSB formation...
  51. Yamamoto K, Kobayashi S, Tsuda M, Kurumizaka H, Takata M, Kono K, et al. Involvement of SLX4 in interstrand cross-link repair is regulated by the Fanconi anemia pathway. Proc Natl Acad Sci U S A. 2011;108:6492-6 pubmed publisher
    ..Stalled replication forks activate the eight-subunit FA core complex, which ubiquitylates FANCD2-FANCI...
  52. Léveillé F, Ferrer M, Medhurst A, Laghmani E, Rooimans M, Bier P, et al. The nuclear accumulation of the Fanconi anemia protein FANCE depends on FANCC. DNA Repair (Amst). 2006;5:556-65 pubmed
    ..component of the nuclear FA core complex, which is required for monoubiquitination of the downstream target FANCD2, an important step in the FA pathway of DNA cross-link repair...
  53. van der Groep P, Hoelzel M, Buerger H, Joenje H, de Winter J, van Diest P. Loss of expression of FANCD2 protein in sporadic and hereditary breast cancer. Breast Cancer Res Treat. 2008;107:41-7 pubmed
    ..Activation of one of the FA proteins (FANCD2) by mono-ubiquitination is an essential step in DNA damage response...
  54. Kalb R, Neveling K, Hoehn H, Schneider H, Linka Y, Batish S, et al. Hypomorphic mutations in the gene encoding a key Fanconi anemia protein, FANCD2, sustain a significant group of FA-D2 patients with severe phenotype. Am J Hum Genet. 2007;80:895-910 pubmed
    b>FANCD2 is an evolutionarily conserved Fanconi anemia (FA) gene that plays a key role in DNA double-strand-type damage responses...