The RIDDLE syndrome protein mediates a ubiquitin-dependent signaling cascade at sites of DNA damage.

PubWeight™: 5.96‹?› | Rank: Top 1%

🔗 View Article (PMID 19203578)

Published in Cell on February 06, 2009

Authors

Grant S Stewart1, Stephanie Panier, Kelly Townsend, Abdallah K Al-Hakim, Nadine K Kolas, Edward S Miller, Shinichiro Nakada, Jarkko Ylanko, Signe Olivarius, Megan Mendez, Ceri Oldreive, Jan Wildenhain, Andrea Tagliaferro, Laurence Pelletier, Nadine Taubenheim, Anne Durandy, Philip J Byrd, Tatjana Stankovic, A Malcolm R Taylor, Daniel Durocher

Author Affiliations

1: Cancer Research UK, Institute for Cancer Studies, University of Birmingham, Edgbaston, Birmingham, UK. g.s.stewart@bham.ac.uk

Articles citing this

(truncated to the top 100)

The DNA damage response: making it safe to play with knives. Mol Cell (2010) 16.13

Dynamics of DNA damage response proteins at DNA breaks: a focus on protein modifications. Genes Dev (2011) 5.60

ATM-dependent chromatin changes silence transcription in cis to DNA double-strand breaks. Cell (2010) 4.73

Mammalian SUMO E3-ligases PIAS1 and PIAS4 promote responses to DNA double-strand breaks. Nature (2009) 4.30

More than just a focus: The chromatin response to DNA damage and its role in genome integrity maintenance. Nat Cell Biol (2011) 4.23

BRCA1 and its toolbox for the maintenance of genome integrity. Nat Rev Mol Cell Biol (2009) 3.79

The SUMO modification pathway is involved in the BRCA1 response to genotoxic stress. Nature (2009) 3.62

A chromatin localization screen reveals poly (ADP ribose)-regulated recruitment of the repressive polycomb and NuRD complexes to sites of DNA damage. Proc Natl Acad Sci U S A (2010) 3.31

BRCA1 tumour suppression occurs via heterochromatin-mediated silencing. Nature (2011) 3.30

Human HDAC1 and HDAC2 function in the DNA-damage response to promote DNA nonhomologous end-joining. Nat Struct Mol Biol (2010) 3.27

Acetylation limits 53BP1 association with damaged chromatin to promote homologous recombination. Nat Struct Mol Biol (2013) 3.18

53BP1-dependent robust localized KAP-1 phosphorylation is essential for heterochromatic DNA double-strand break repair. Nat Cell Biol (2010) 2.88

Mechanisms of double-strand break repair in somatic mammalian cells. Biochem J (2009) 2.68

53BP1 is a reader of the DNA-damage-induced H2A Lys 15 ubiquitin mark. Nature (2013) 2.64

Non-canonical inhibition of DNA damage-dependent ubiquitination by OTUB1. Nature (2010) 2.47

Regulation of DNA end joining, resection, and immunoglobulin class switch recombination by 53BP1. Mol Cell (2011) 2.46

Ubiquitin signalling in DNA replication and repair. Nat Rev Mol Cell Biol (2010) 2.41

HERC2 coordinates ubiquitin-dependent assembly of DNA repair factors on damaged chromosomes. Nat Cell Biol (2009) 2.37

RNF8- and RNF168-dependent degradation of KDM4A/JMJD2A triggers 53BP1 recruitment to DNA damage sites. EMBO J (2012) 2.34

DNA damage signaling in response to double-strand breaks during mitosis. J Cell Biol (2010) 2.30

The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks. Nat Struct Mol Biol (2011) 2.23

Histone H1 couples initiation and amplification of ubiquitin signalling after DNA damage. Nature (2015) 2.16

RNF4, a SUMO-targeted ubiquitin E3 ligase, promotes DNA double-strand break repair. Genes Dev (2012) 2.16

The ubiquitin-selective segregase VCP/p97 orchestrates the response to DNA double-strand breaks. Nat Cell Biol (2011) 2.15

Structural basis for specific recognition of Lys 63-linked polyubiquitin chains by tandem UIMs of RAP80. EMBO J (2009) 2.14

The NuRD chromatin-remodeling complex regulates signaling and repair of DNA damage. J Cell Biol (2010) 2.06

Repair of strand breaks by homologous recombination. Cold Spring Harb Perspect Biol (2013) 1.96

PRP19 transforms into a sensor of RPA-ssDNA after DNA damage and drives ATR activation via a ubiquitin-mediated circuitry. Mol Cell (2013) 1.95

Fluorescence-based sensors to monitor localization and functions of linear and K63-linked ubiquitin chains in cells. Mol Cell (2012) 1.94

The BRCA1-RAP80 complex regulates DNA repair mechanism utilization by restricting end resection. J Biol Chem (2011) 1.91

SUMO-targeted ubiquitin E3 ligase RNF4 is required for the response of human cells to DNA damage. Genes Dev (2012) 1.90

A mitotic phosphorylation feedback network connects Cdk1, Plk1, 53BP1, and Chk2 to inactivate the G(2)/M DNA damage checkpoint. PLoS Biol (2010) 1.90

A cooperative activation loop among SWI/SNF, gamma-H2AX and H3 acetylation for DNA double-strand break repair. EMBO J (2010) 1.86

The ubiquitin landscape at DNA double-strand breaks. J Cell Biol (2009) 1.86

The chromatin-remodeling factor CHD4 coordinates signaling and repair after DNA damage. J Cell Biol (2010) 1.82

PR-Set7 and H4K20me1: at the crossroads of genome integrity, cell cycle, chromosome condensation, and transcription. Genes Dev (2012) 1.81

The p400 ATPase regulates nucleosome stability and chromatin ubiquitination during DNA repair. J Cell Biol (2010) 1.80

A genome-scale DNA repair RNAi screen identifies SPG48 as a novel gene associated with hereditary spastic paraplegia. PLoS Biol (2010) 1.77

A viral E3 ligase targets RNF8 and RNF168 to control histone ubiquitination and DNA damage responses. EMBO J (2010) 1.75

53BP1: pro choice in DNA repair. Trends Cell Biol (2013) 1.75

BMI1-mediated histone ubiquitylation promotes DNA double-strand break repair. J Cell Biol (2010) 1.74

Chromatin and the genome integrity network. Nat Rev Genet (2013) 1.72

The mechanism of OTUB1-mediated inhibition of ubiquitination. Nature (2012) 1.70

The E3 ligase RNF8 regulates KU80 removal and NHEJ repair. Nat Struct Mol Biol (2012) 1.64

Chromatin dynamics and the repair of DNA double strand breaks. Cell Cycle (2011) 1.63

OTUB1 co-opts Lys48-linked ubiquitin recognition to suppress E2 enzyme function. Mol Cell (2012) 1.58

RNF8-dependent histone modifications regulate nucleosome removal during spermatogenesis. Dev Cell (2010) 1.57

Focus on histone variant H2AX: to be or not to be. FEBS Lett (2010) 1.56

A two-step mechanism for TRF2-mediated chromosome-end protection. Nature (2013) 1.55

BMI1 is recruited to DNA breaks and contributes to DNA damage-induced H2A ubiquitination and repair. Mol Cell Biol (2011) 1.52

REV7 counteracts DNA double-strand break resection and affects PARP inhibition. Nature (2015) 1.50

Assembly of checkpoint and repair machineries at DNA damage sites. Trends Biochem Sci (2009) 1.49

Modulation of DNA damage and repair pathways by human tumour viruses. Viruses (2015) 1.49

DNA-damage response and repair activities at uncapped telomeres depend on RNF8. Nat Cell Biol (2011) 1.49

DNA damage response: three levels of DNA repair regulation. Cold Spring Harb Perspect Biol (2013) 1.48

A novel ubiquitin mark at the N-terminal tail of histone H2As targeted by RNF168 ubiquitin ligase. Cell Cycle (2012) 1.46

53BP1, BRCA1, and the choice between recombination and end joining at DNA double-strand breaks. Mol Cell Biol (2014) 1.45

Crosstalk between chromatin state and DNA damage response in cellular senescence and cancer. Nat Rev Cancer (2012) 1.45

The proteasomal de-ubiquitinating enzyme POH1 promotes the double-strand DNA break response. EMBO J (2012) 1.44

Guidelines for genetic studies in single patients: lessons from primary immunodeficiencies. J Exp Med (2014) 1.41

RNF4-dependent hybrid SUMO-ubiquitin chains are signals for RAP80 and thereby mediate the recruitment of BRCA1 to sites of DNA damage. Sci Signal (2012) 1.40

Rnf8 deficiency impairs class switch recombination, spermatogenesis, and genomic integrity and predisposes for cancer. J Exp Med (2010) 1.40

Human RNF169 is a negative regulator of the ubiquitin-dependent response to DNA double-strand breaks. J Cell Biol (2012) 1.37

The intrinsic antiviral defense to incoming HSV-1 genomes includes specific DNA repair proteins and is counteracted by the viral protein ICP0. PLoS Pathog (2011) 1.37

RNF168, a new RING finger, MIU-containing protein that modifies chromatin by ubiquitination of histones H2A and H2AX. BMC Mol Biol (2009) 1.34

Class switching and meiotic defects in mice lacking the E3 ubiquitin ligase RNF8. J Exp Med (2010) 1.33

The MMS22L-TONSL complex mediates recovery from replication stress and homologous recombination. Mol Cell (2010) 1.32

DNA DSB repair pathway choice: an orchestrated handover mechanism. Br J Radiol (2014) 1.31

Double strand break repair functions of histone H2AX. Mutat Res (2013) 1.31

The USP1/UAF1 complex promotes double-strand break repair through homologous recombination. Mol Cell Biol (2011) 1.31

Push back to respond better: regulatory inhibition of the DNA double-strand break response. Nat Rev Mol Cell Biol (2013) 1.29

Monoubiquitination of H2AX protein regulates DNA damage response signaling. J Biol Chem (2011) 1.29

Links between genome integrity and BRCA1 tumor suppression. Trends Biochem Sci (2012) 1.26

Sensitivity to poly(ADP-ribose) polymerase (PARP) inhibition identifies ubiquitin-specific peptidase 11 (USP11) as a regulator of DNA double-strand break repair. J Biol Chem (2010) 1.26

A new non-catalytic role for ubiquitin ligase RNF8 in unfolding higher-order chromatin structure. EMBO J (2012) 1.24

The RNF8/RNF168 ubiquitin ligase cascade facilitates class switch recombination. Proc Natl Acad Sci U S A (2009) 1.24

BBAP monoubiquitylates histone H4 at lysine 91 and selectively modulates the DNA damage response. Mol Cell (2009) 1.23

Genomic instability, defective spermatogenesis, immunodeficiency, and cancer in a mouse model of the RIDDLE syndrome. PLoS Genet (2011) 1.23

DNA damage-inducible SUMOylation of HERC2 promotes RNF8 binding via a novel SUMO-binding Zinc finger. J Cell Biol (2012) 1.22

The Lys63-specific deubiquitinating enzyme BRCC36 is regulated by two scaffold proteins localizing in different subcellular compartments. J Biol Chem (2010) 1.22

Homozygous deficiency of ubiquitin-ligase ring-finger protein RNF168 mimics the radiosensitivity syndrome of ataxia-telangiectasia. Cell Death Differ (2011) 1.21

The deubiquitylating enzyme USP44 counteracts the DNA double-strand break response mediated by the RNF8 and RNF168 ubiquitin ligases. J Biol Chem (2013) 1.20

Primary antibody deficiencies. Nat Rev Immunol (2013) 1.20

UMI, a novel RNF168 ubiquitin binding domain involved in the DNA damage signaling pathway. Mol Cell Biol (2010) 1.18

CBX4-mediated SUMO modification regulates BMI1 recruitment at sites of DNA damage. Nucleic Acids Res (2012) 1.16

Give me a break, but not in mitosis: the mitotic DNA damage response marks DNA double-strand breaks with early signaling events. Cell Cycle (2011) 1.16

MDC1: The art of keeping things in focus. Chromosoma (2010) 1.15

Pathway choice in DNA double strand break repair: observations of a balancing act. Genome Integr (2012) 1.13

The increasing complexity of the ubiquitin code. Nat Cell Biol (2016) 1.13

Differential regulation of JAMM domain deubiquitinating enzyme activity within the RAP80 complex. J Biol Chem (2010) 1.12

USP51 deubiquitylates H2AK13,15ub and regulates DNA damage response. Genes Dev (2016) 1.12

Nucleosome acidic patch promotes RNF168- and RING1B/BMI1-dependent H2AX and H2A ubiquitination and DNA damage signaling. PLoS Genet (2014) 1.10

The ATM signaling network in development and disease. Front Genet (2013) 1.09

Prediction of breast cancer sensitivity to neoadjuvant chemotherapy based on status of DNA damage repair proteins. Breast Cancer Res (2010) 1.09

The nucleosome acidic patch plays a critical role in RNF168-dependent ubiquitination of histone H2A. Nat Commun (2014) 1.07

Nucleoporin NUP153 guards genome integrity by promoting nuclear import of 53BP1. Cell Death Differ (2011) 1.07

USP3 counteracts RNF168 via deubiquitinating H2A and γH2AX at lysine 13 and 15. Cell Cycle (2013) 1.07

The RNA-binding protein fused in sarcoma (FUS) functions downstream of poly(ADP-ribose) polymerase (PARP) in response to DNA damage. J Biol Chem (2013) 1.07

H2AX post-translational modifications in the ionizing radiation response and homologous recombination. Cell Cycle (2010) 1.06

Viral E3 ubiquitin ligase-mediated degradation of a cellular E3: viral mimicry of a cellular phosphorylation mark targets the RNF8 FHA domain. Mol Cell (2012) 1.06

Articles by these authors

Systematic identification of protein complexes in Saccharomyces cerevisiae by mass spectrometry. Nature (2002) 37.66

The chemical genomic portrait of yeast: uncovering a phenotype for all genes. Science (2008) 8.52

Orchestration of the DNA-damage response by the RNF8 ubiquitin ligase. Science (2007) 7.61

RNF168 binds and amplifies ubiquitin conjugates on damaged chromosomes to allow accumulation of repair proteins. Cell (2009) 6.89

MDC1 is a mediator of the mammalian DNA damage checkpoint. Nature (2003) 6.81

BAC TransgeneOmics: a high-throughput method for exploration of protein function in mammals. Nat Methods (2008) 6.51

Cernunnos, a novel nonhomologous end-joining factor, is mutated in human immunodeficiency with microcephaly. Cell (2006) 5.33

Systematic analysis of human protein complexes identifies chromosome segregation proteins. Science (2010) 4.69

The CRAPome: a contaminant repository for affinity purification-mass spectrometry data. Nat Methods (2013) 4.34

A phosphatase complex that dephosphorylates gammaH2AX regulates DNA damage checkpoint recovery. Nature (2005) 4.33

Genome-scale RNAi profiling of cell division in human tissue culture cells. Nat Cell Biol (2007) 3.91

Protein phosphatase 2A protects centromeric sister chromatid cohesion during meiosis I. Nature (2006) 3.81

Human uracil-DNA glycosylase deficiency associated with profoundly impaired immunoglobulin class-switch recombination. Nat Immunol (2003) 3.81

AID mutant analyses indicate requirement for class-switch-specific cofactors. Nat Immunol (2003) 3.69

An endoribonuclease-prepared siRNA screen in human cells identifies genes essential for cell division. Nature (2004) 3.69

A cell cycle-dependent regulatory circuit composed of 53BP1-RIF1 and BRCA1-CtIP controls DNA repair pathway choice. Mol Cell (2013) 3.55

Efficacy of gene therapy for X-linked severe combined immunodeficiency. N Engl J Med (2010) 3.54

Regulation of DNA damage responses by ubiquitin and SUMO. Mol Cell (2013) 3.43

Phosphoinositide 3-kinase δ gene mutation predisposes to respiratory infection and airway damage. Science (2013) 3.33

Mitosis inhibits DNA double-strand break repair to guard against telomere fusions. Science (2014) 3.31

Centriole assembly requires both centriolar and pericentriolar material proteins. Dev Cell (2004) 3.23

The time course and chromosomal localization of recombination-related proteins at meiosis in the mouse are compatible with models that can resolve the early DNA-DNA interactions without reciprocal recombination. J Cell Sci (2002) 3.07

ICOS deficiency is associated with a severe reduction of CXCR5+CD4 germinal center Th cells. J Immunol (2006) 2.99

HAUS, the 8-subunit human Augmin complex, regulates centrosome and spindle integrity. Curr Biol (2009) 2.98

Mcl-1 expression has in vitro and in vivo significance in chronic lymphocytic leukemia and is associated with other poor prognostic markers. Blood (2008) 2.88

Chemical genetics reveals a complex functional ground state of neural stem cells. Nat Chem Biol (2007) 2.88

The AP-1A and AP-1B clathrin adaptor complexes define biochemically and functionally distinct membrane domains. J Cell Biol (2003) 2.78

A genome-wide screen identifies the evolutionarily conserved KEOPS complex as a telomere regulator. Cell (2006) 2.71

Independent and sequential recruitment of NHEJ and HR factors to DNA damage sites in mammalian cells. J Cell Biol (2005) 2.64

53BP1 is a reader of the DNA-damage-induced H2A Lys 15 ubiquitin mark. Nature (2013) 2.64

RNA interference rescue by bacterial artificial chromosome transgenesis in mammalian tissue culture cells. Proc Natl Acad Sci U S A (2005) 2.59

Aurora A phosphorylation of TACC3/maskin is required for centrosome-dependent microtubule assembly in mitosis. J Cell Biol (2005) 2.57

Human TRAF3 adaptor molecule deficiency leads to impaired Toll-like receptor 3 response and susceptibility to herpes simplex encephalitis. Immunity (2010) 2.47

Non-canonical inhibition of DNA damage-dependent ubiquitination by OTUB1. Nature (2010) 2.47

An allosteric inhibitor of the human Cdc34 ubiquitin-conjugating enzyme. Cell (2011) 2.35

Linking promoters to functional transcripts in small samples with nanoCAGE and CAGEscan. Nat Methods (2010) 2.30

Inactivation of Exonuclease 1 in mice results in DNA mismatch repair defects, increased cancer susceptibility, and male and female sterility. Genes Dev (2003) 2.28

Xrcc4 physically links DNA end processing by polynucleotide kinase to DNA ligation by DNA ligase IV. EMBO J (2004) 2.24

Constitutive phosphorylation of MDC1 physically links the MRE11-RAD50-NBS1 complex to damaged chromatin. J Cell Biol (2008) 2.21

Saccharomyces cerevisiae Rad9 acts as a Mec1 adaptor to allow Rad53 activation. Curr Biol (2005) 2.20

Subdiffraction imaging of centrosomes reveals higher-order organizational features of pericentriolar material. Nat Cell Biol (2012) 2.19

X-linked susceptibility to mycobacteria is caused by mutations in NEMO impairing CD40-dependent IL-12 production. J Exp Med (2006) 2.18

Regulatory ubiquitylation in response to DNA double-strand breaks. DNA Repair (Amst) (2009) 2.17

Specific recruitment of human cohesin to laser-induced DNA damage. J Biol Chem (2002) 2.13

The PARP inhibitor olaparib induces significant killing of ATM-deficient lymphoid tumor cells in vitro and in vivo. Blood (2010) 2.09

PP4 is a gamma H2AX phosphatase required for recovery from the DNA damage checkpoint. EMBO Rep (2008) 2.09

The molecular architecture of the mammalian DNA repair enzyme, polynucleotide kinase. Mol Cell (2005) 2.09

Significant conservation of synthetic lethal genetic interaction networks between distantly related eukaryotes. Proc Natl Acad Sci U S A (2008) 2.01

Structural and functional versatility of the FHA domain in DNA-damage signaling by the tumor suppressor kinase Chk2. Mol Cell (2002) 1.99

MRE11 promotes AKT phosphorylation in direct response to DNA double-strand breaks. Cell Cycle (2011) 1.97

The ubiquitous role of ubiquitin in the DNA damage response. DNA Repair (Amst) (2010) 1.94

The mammalian SPD-2 ortholog Cep192 regulates centrosome biogenesis. Curr Biol (2008) 1.88

Elg1 forms an alternative PCNA-interacting RFC complex required to maintain genome stability. Curr Biol (2003) 1.88

Systematic identification of fragile sites via genome-wide location analysis of gamma-H2AX. Nat Struct Mol Biol (2010) 1.87

The ataxia-oculomotor apraxia 1 gene product has a role distinct from ATM and interacts with the DNA strand break repair proteins XRCC1 and XRCC4. DNA Repair (Amst) (2004) 1.86

Combinations of antibiotics and nonantibiotic drugs enhance antimicrobial efficacy. Nat Chem Biol (2011) 1.84

Mutation status of the residual ATM allele is an important determinant of the cellular response to chemotherapy and survival in patients with chronic lymphocytic leukemia containing an 11q deletion. J Clin Oncol (2007) 1.82

Mutations in the ATM gene lead to impaired overall and treatment-free survival that is independent of IGVH mutation status in patients with B-CLL. Blood (2005) 1.81

RIDDLE immunodeficiency syndrome is linked to defects in 53BP1-mediated DNA damage signaling. Proc Natl Acad Sci U S A (2007) 1.79

A viral E3 ligase targets RNF8 and RNF168 to control histone ubiquitination and DNA damage responses. EMBO J (2010) 1.75

The genetic network controlling plasma cell differentiation. Semin Immunol (2011) 1.66

Human ICOS deficiency abrogates the germinal center reaction and provides a monogenic model for common variable immunodeficiency. Blood (2005) 1.63

Tandem protein interaction modules organize the ubiquitin-dependent response to DNA double-strand breaks. Mol Cell (2012) 1.62

Transferrin receptor recycling in the absence of perinuclear recycling endosomes. J Cell Biol (2002) 1.62

OTUB1 co-opts Lys48-linked ubiquitin recognition to suppress E2 enzyme function. Mol Cell (2012) 1.58

Golgin tethers define subpopulations of COPI vesicles. Science (2005) 1.54

PFI-1, a highly selective protein interaction inhibitor, targeting BET Bromodomains. Cancer Res (2013) 1.53

Cross-species discovery of syncretic drug combinations that potentiate the antifungal fluconazole. Mol Syst Biol (2011) 1.51

Essential role of Fkbp6 in male fertility and homologous chromosome pairing in meiosis. Science (2003) 1.51

Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency. Clin Immunol (2004) 1.51

Atomic structure of the KEOPS complex: an ancient protein kinase-containing molecular machine. Mol Cell (2008) 1.51

Regulation of DNA-end resection by hnRNPU-like proteins promotes DNA double-strand break signaling and repair. Mol Cell (2012) 1.50

Problems with co-funding in Canada. Science (2005) 1.48

APLF (C2orf13) facilitates nonhomologous end-joining and undergoes ATM-dependent hyperphosphorylation following ionizing radiation. DNA Repair (Amst) (2008) 1.44

Activation-induced cytidine deaminase (AID) is required for B-cell tolerance in humans. Proc Natl Acad Sci U S A (2011) 1.44

A strategy for modulation of enzymes in the ubiquitin system. Science (2013) 1.43

The block in immunoglobulin class switch recombination caused by activation-induced cytidine deaminase deficiency occurs prior to the generation of DNA double strand breaks in switch mu region. J Immunol (2003) 1.43

A human immunodeficiency caused by mutations in the PIK3R1 gene. J Clin Invest (2014) 1.42

A predictive model for drug bioaccumulation and bioactivity in Caenorhabditis elegans. Nat Chem Biol (2010) 1.41