2021
Fanci-FANCD2 Promotes Genome Stability and DNA Repair By Down-Regulating BLM Helicase Activity
Liang F, Nagarajan A, Pillai M, Sung P, Kupfer G. Fanci-FANCD2 Promotes Genome Stability and DNA Repair By Down-Regulating BLM Helicase Activity. Blood 2021, 138: 1113. DOI: 10.1182/blood-2021-152218.Peer-Reviewed Original ResearchDNA end resectionReplication forksGenome stabilityDNA repairResection activityRAD51 recruitmentEnd resectionAmino acid deletionBloom syndromeFanconi anemiaMutant cellsFA DNA repair pathwayAcid deletionDNA damageDNA damage hypersensitivityReplication fork collapseBLM helicase activityEfficient DNA repairHolliday junction dissolutionDNA damage repairDNA repair pathwaysRAD51 foci formationDouble Holliday junction dissolutionIsolation of proteinsHU sensitivity
2019
DNA requirement in FANCD2 deubiquitination by USP1-UAF1-RAD51AP1 in the Fanconi anemia DNA damage response
Liang F, Miller AS, Longerich S, Tang C, Maranon D, Williamson EA, Hromas R, Wiese C, Kupfer GM, Sung P. DNA requirement in FANCD2 deubiquitination by USP1-UAF1-RAD51AP1 in the Fanconi anemia DNA damage response. Nature Communications 2019, 10: 2849. PMID: 31253762, PMCID: PMC6599204, DOI: 10.1038/s41467-019-10408-5.Peer-Reviewed Original ResearchConceptsFANCD2 deubiquitinationDNA damage response pathwayFanconi anemiaUSP1-UAF1 complexDNA damage toleranceDNA damage responseDamage response pathwayImportance of DNAGenome repairDamage responseResponse pathwaysUAF1DNA requirementsCellular settingsFA pathwayDNA bindingDownstream eventsDeubiquitinationRAD51AP1DNA crosslinksBiochemical systemsBone marrow failureChromosomal lesionsMultigenic diseasesMultifaceted roleBinding of FANCI-FANCD2 Complex to RNA and R-Loops Stimulates Robust FANCD2 Monoubiquitination
Liang Z, Liang F, Teng Y, Chen X, Liu J, Longerich S, Rao T, Green AM, Collins NB, Xiong Y, Lan L, Sung P, Kupfer GM. Binding of FANCI-FANCD2 Complex to RNA and R-Loops Stimulates Robust FANCD2 Monoubiquitination. Cell Reports 2019, 26: 564-572.e5. PMID: 30650351, PMCID: PMC6350941, DOI: 10.1016/j.celrep.2018.12.084.Peer-Reviewed Original ResearchConceptsR-loopsCo-transcriptional R-loopsFANCI-FANCD2 complexFanconi anemiaR-loop structuresSeries of RNADNA replicative stressGuanine-rich sequencesFANCD2 monoubiquitinationGenomic lociID2 complexReplicative stressFA cellsRNA transcriptionFA pathwayComplex bindsDNA hybridsCancer predispositionBone marrow failureDNA damageRNAMonoubiquitinationDevelopmental abnormalitiesMarrow failureSsRNA
2016
The Role of UAF1 in the Fanconi Anemia Pathway Regulation of Homologous Recombination-Mediated Genome Maintenance
Liang F, Longerich S, Tang C, Buzovestsky O, Xiong Y, Maranon D, Wiese C, Miller A, Sung P, Kupfer G. The Role of UAF1 in the Fanconi Anemia Pathway Regulation of Homologous Recombination-Mediated Genome Maintenance. Blood 2016, 128: 1041. DOI: 10.1182/blood.v128.22.1041.1041.Peer-Reviewed Original ResearchHomologous recombinationGenome maintenanceDNA damageFA DNA repair pathwayCancer-prone genetic diseasesFanconi anemiaDNA damage hypersensitivityFunctional synergySynaptic complex assemblyProtein complex formationRad51 presynaptic filamentD-loop reactionDNA repair functionDNA damage repairDNA binding activityCell linesFANCD2 ubiquitinationGenome stabilityComplex formationRAD51 recombinaseHuman cell linesBinds DNAPresynaptic filamentRecombinase RAD51UAF1