179:
has methods to repair at every stage of the cell cycle. There are multiple different repair pathways but the FA pathway is the one that involves the FANC proteins. When cross-link is detected, then the ataxia-telangiectasia and RAD3-related protein will mediate the phosphorylation (P) of the FA core complex. This phosphorylated FA core complex is what is required to have a successful monoubiquitination of the two components that form the FANCI–D2 complex. Each of the proteins of the FA core complex are needed for this phosphorylation step except for FANCM. When a typical cell senses DNA damage it targets the monoubiquitinated isoform of FANCI–D2 to the chromatid with DNA damage, which is the cross-link. Studies have also shown that there is a connection between the FA DNA repair pathway and stem cell regulation but it is still unclear. FANC proteins also play a role in redox signaling and repair of oxidative DNA damages. Recent studies have dove into the FANC protein, FANCJ, and its enzymatic function along with its roles in repair. Other studies have shown the correlation between the FANC pathway and multiple other protein post translational modifications from ubiquitin-like families.
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proteins has been associated with a range of conditions, including the rendering of cell hypersensitivity to a type of DNA damage known as DNA interstrand cross-links (ICL) and defective DNA repair. FANC protein mutations have also lead to reduced fertility and predisposition to cancers like breast cancer and myeloid leukaemia. FANC proteins FANCD1 (BRCA2), FANCJ (BRIP), and FANCN (PALB2) have even been identified as the breast cancer susceptibility proteins. If a cell were to lack the FANC gene to code for these proteins then the cell would show a hypersensitive phenotype following H2O2 treatment.
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1376:
187:
A mutation in 13 FANC genes can result in
Fanconi anemia (FA), which is a cancer-prone chromosome instability disorder. Fanconi anemia occurs when there is a biallelic mutation that inactivates the genes that are in charge of the replication stress associated DNA damage response. Dysfunction of FANC
84:
and the accessory proteins (FAAP20, FAAP24, and FAAP100). These accessory proteins are also called
Fanconi anemia associated proteins (FAAPs). There is also a group called the anchor complex which consists of FANCM, FAAP24, MHF1 (FAAP16/ CENP-S), and MHF2 (FAAP10/ CENP-X). The FANC proteins that are
178:
and damage response. FANC proteins are also in charge of repairing complex DNA interstrand cross-linking lesions and maintaining the genomic stability during DNA replication. DNA cross-linking is what hinders transcription and replication from occurring in the cell so it is important that the cell
43:
The FA genes that code for the FANC proteins are a part of the caretaker group of cancer genes that prevent the buildup of mutations and chromosome abnormalities. The multiple FANC proteins come together to add up to the FANC/BRCA pathway.
34:
Fanconi anemia was first described in 1927 by Guido
Fanconi, a Swiss pediatrician. It is a chromosome instability syndrome characterized by the progressiveness of bone marrow failure and of cancer proneness.
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There are a large number of FANC proteins that participate in the FA pathway. It has a nuclear complex also known as the ‘FA core complex’ which is formed by the interaction of
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Naim V, Rosselli F (June 2009). "The FANC pathway and BLM collaborate during mitosis to prevent micro-nucleation and chromosome abnormalities".
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427:"Cellular and molecular consequences of defective Fanconi anemia proteins in replication-coupled DNA repair: Mechanistic insights"
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855:"BLM and the FANC proteins collaborate in a common pathway in response to stalled replication forks"
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701:"Oxidative Stress/Damage Induces Multimerization and Interaction of Fanconi Anemia Proteins"
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are a network of at least 15 proteins that are associated with a cell process known as the
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752:"FAN1 Acts with FANCI-FANCD2 to Promote DNA Interstrand Cross-Link Repair"
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Mutation
Research/Fundamental and Molecular Mechanisms of Mutagenesis
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Primary immunodeficiency diseases: a molecular and genetic approach
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FANC proteins are required to promote BLM-mediated anaphase.
809:"The ubiquitin family meets the Fanconi anemia proteins"
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Hans D. Ochs; C. I. Edvard Smith; Jennifer Puck (2007).
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653:"The FANC pathway and mitosis: a replication legacy"
604:"The FANC pathway and mitosis: A replication legacy"
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813:Mutation Research/Reviews in Mutation Research
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390:. Oxford University Press US. pp. 431–.
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327:at the U.S. National Library of Medicine
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651:Naim V, Rosselli F (September 2009).
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276:FANC protein FANCD1 is also known as
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304:FANC protein FANCP is also known as
297:FANC protein FANCO is also known as
290:FANC protein FANCN is also known as
283:FANC protein FANCJ is also known as
705:The Journal of Biological Chemistry
85:not a part of the core complex are
1229:Proliferating Cell Nuclear Antigen
1160:Microhomology-mediated end joining
248:FANC proteins also interacts with
241:FANC proteins also interacts with
234:FANC proteins also interacts with
227:FANC proteins also interacts with
220:FANC proteins also interacts with
213:FANC proteins also interacts with
206:FANC proteins also interacts with
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1244:Meiotic recombination checkpoint
1:
269:FANCC protein interactS with
262:FANCC protein interacts with
255:FANCC protein interacts with
196:FANC proteins are related to
1390:. You can help Knowledge by
1126:Transcription-coupled repair
443:10.1016/j.mrfmmm.2009.02.003
825:10.1016/j.mrrev.2016.06.004
1463:
1369:
1148:Non-homologous end joining
972:Nucleotide excision repair
965:Poly ADP ribose polymerase
853:Pichierri, Pietro (2004).
1346:
807:Renaudin, Xavier (2016).
1143:Homology directed repair
1065:Homologous recombination
871:10.1038/sj.emboj.7600277
537:"Fanconi Anemia Protein"
425:Thompson, Larry (2009).
329:Medical Subject Headings
192:Similar/ Related Protein
777:10.1126/science.1192656
1386:-related article is a
1165:Postreplication repair
960:Uracil-DNA glycosylase
718:10.1074/jbc.M403527200
699:Park, Su-Jung (2004).
602:Naim, Valeria (2009).
174:They are involved in
1271:core protein complex
946:Base excision repair
670:10.4161/cc.8.18.9538
621:10.4161/cc.8.18.9538
105:core protein complex
100:Components include:
1043:DNA mismatch repair
768:2010Sci...329..693L
711:(29): 30053–30059.
750:Ting, Liu (2010).
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1239:Adaptive response
865:(15): 3154–3163.
762:(5992): 693–696.
614:(18): 2907–2912.
397:978-0-19-514774-2
16:Group of proteins
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1442:Protein families
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1212:Other/ungrouped
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955:DNA glycosylase
938:Excision repair
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663:(18): 2907–11.
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176:DNA replication
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1119:Other pathways
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437:(1–2): 54–72.
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343:Nat. Cell Biol
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24:Fanconi anemia
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1447:Protein stubs
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1267:FANC proteins
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1088:RecQ helicase
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325:FANC+Proteins
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20:FANC proteins
1392:expanding it
1381:
1366:
1270:
1266:
1251:DNA helicase
1234:8-Oxoguanine
1203:SOS response
1083:RecF pathway
1036:Excinuclease
862:
858:
848:
836:. Retrieved
816:
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730:. Retrieved
708:
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574:. Retrieved
570:
567:"FANCA gene"
544:. Retrieved
540:
434:
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401:. Retrieved
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379:
349:(6): 761–8.
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183:Pathogenesis
173:
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33:
19:
18:
838:28 November
732:28 November
576:28 November
571:Medlineplus
546:28 November
403:21 December
1437:DNA repair
1431:Categories
1191:Regulation
1170:Photolyase
930:DNA repair
657:Cell Cycle
608:Cell Cycle
535:Gupta, S.
312:References
48:Components
39:Properties
1011:XPG/ERCC5
996:XPD/ERCC2
819:: 36–46.
794:206527789
1356:Category
1006:XPF/DDB1
1001:XPE/DDB1
889:15257300
833:27543315
786:20671156
727:15138265
679:19729998
638:37042438
630:19729998
461:19622404
363:19465921
222:DNA-PKcs
170:Function
1384:protein
1198:SOS box
950:AP site
764:Bibcode
756:Science
452:2714807
142:other:
30:History
1323:FANCD2
1318:FANCD1
1078:RecBCD
1031:RAD23B
1026:RAD23A
887:
880:514912
877:
831:
792:
784:
725:
677:
636:
628:
459:
449:
394:
371:330040
369:
361:
331:(MeSH)
299:RAD51C
148:FANCD2
144:FANCD1
93:, and
87:FANCD1
1382:This
1338:FANCN
1333:FANCJ
1328:FANCI
1311:FANCM
1306:FANCL
1301:FANCG
1296:FANCF
1291:FANCE
1286:FANCC
1281:FANCB
1276:FANCA
1136:ERCC8
1131:ERCC6
1095:RAD51
1016:ERCC1
790:S2CID
634:S2CID
367:S2CID
292:PALB2
285:BRIP1
278:BRCA2
208:BRCA1
164:FANCP
160:FANCN
156:FANCJ
152:FANCI
137:FANCM
133:FANCL
129:FANCG
125:FANCF
121:FANCE
117:FANCC
113:FANCB
109:FANCA
95:FANCN
91:FANCJ
82:FANCM
78:FANCL
74:FANCG
70:FANCF
66:FANCE
62:FANCC
58:FANCB
54:FANCA
1388:stub
1224:PcrA
1180:CRY2
1175:CRY1
1110:LexA
1105:Slx4
1100:Sgs1
1073:RecA
1053:MSH2
1048:MLH1
976:ERCC
885:PMID
840:2023
829:PMID
782:PMID
734:2023
723:PMID
675:PMID
626:PMID
578:2023
548:2023
457:PMID
405:2010
392:ISBN
359:PMID
306:SLX4
257:cdc2
243:FAN1
236:Ku80
229:Ku70
215:LIG4
198:BRCA
26:.
1260:WRN
1255:BLM
1219:Ogt
1021:RPA
991:XPC
986:XPB
981:XPA
875:PMC
867:doi
821:doi
817:769
772:doi
760:329
713:doi
709:279
665:doi
616:doi
447:PMC
439:doi
435:668
351:doi
273:.
271:p53
266:.
264:PKR
252:.
250:XPF
1433::
1269::
1253::
1153:Ku
883:.
873:.
863:23
861:.
857:.
827:.
815:.
811:.
788:.
780:.
770:.
758:.
754:.
742:^
721:.
707:.
703:.
687:^
673:.
659:.
655:.
632:.
624:.
610:.
606:.
586:^
569:.
556:^
539:.
469:^
455:.
445:.
433:.
429:.
413:^
365:.
357:.
347:11
345:.
308:.
301:.
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224:.
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210:.
200:.
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