Published in DNA Repair (Amst) on July 31, 2010
The spatial organization of non-homologous end joining: from bridging to end joining. DNA Repair (Amst) (2014) 0.99
Functional analysis of naturally occurring DCLRE1C mutations and correlation with the clinical phenotype of ARTEMIS deficiency. J Allergy Clin Immunol (2015) 0.79
DCLRE1C (ARTEMIS) mutations causing phenotypes ranging from atypical severe combined immunodeficiency to mere antibody deficiency. Hum Mol Genet (2015) 0.78
Evaluation of Severe Combined Immunodeficiency and Combined Immunodeficiency Pediatric Patients on the Basis of Cellular Radiosensitivity. J Mol Diagn (2015) 0.77
Autoinhibition of the Nuclease ARTEMIS is Mediated by a Physical Interaction between Its Catalytic and C-terminal Domains. J Biol Chem (2017) 0.75
Unsolved mystery: the role of BRCA1 in DNA end-joining. J Radiat Res (2016) 0.75
Possible role of Artemis c.512C>G polymorphic variant in Omenn syndrome. DNA Repair (Amst) (2010) 0.75
Germline mutations predisposing to diffuse large B-cell lymphoma. Blood Cancer J (2017) 0.75
ATM signaling facilitates repair of DNA double-strand breaks associated with heterochromatin. Mol Cell (2008) 6.60
ATM and DNA-PK function redundantly to phosphorylate H2AX after exposure to ionizing radiation. Cancer Res (2004) 6.38
Sustained correction of X-linked severe combined immunodeficiency by ex vivo gene therapy. N Engl J Med (2002) 6.25
A splicing mutation affecting expression of ataxia-telangiectasia and Rad3-related protein (ATR) results in Seckel syndrome. Nat Genet (2003) 6.03
Crystal structure of an Hsp90-nucleotide-p23/Sba1 closed chaperone complex. Nature (2006) 5.42
A pathway of double-strand break rejoining dependent upon ATM, Artemis, and proteins locating to gamma-H2AX foci. Mol Cell (2004) 5.29
Chaperoned ubiquitylation--crystal structures of the CHIP U box E3 ubiquitin ligase and a CHIP-Ubc13-Uev1a complex. Mol Cell (2005) 4.05
The 3D structure of the immunoglobulin heavy-chain locus: implications for long-range genomic interactions. Cell (2008) 3.92
Activation of the ATPase activity of hsp90 by the stress-regulated cochaperone aha1. Mol Cell (2002) 3.89
gammaH2AX foci analysis for monitoring DNA double-strand break repair: strengths, limitations and optimization. Cell Cycle (2010) 3.56
The impact of a negligent G2/M checkpoint on genomic instability and cancer induction. Nat Rev Cancer (2007) 3.54
The role of double-strand break repair - insights from human genetics. Nat Rev Genet (2006) 3.25
A robust model for read count data in exome sequencing experiments and implications for copy number variant calling. Bioinformatics (2012) 3.10
Chk2 is a tumor suppressor that regulates apoptosis in both an ataxia telangiectasia mutated (ATM)-dependent and an ATM-independent manner. Mol Cell Biol (2002) 3.08
Mutations in pericentrin cause Seckel syndrome with defective ATR-dependent DNA damage signaling. Nat Genet (2007) 3.08
ATR-dependent phosphorylation and activation of ATM in response to UV treatment or replication fork stalling. EMBO J (2006) 3.05
Primary B cell immunodeficiencies: comparisons and contrasts. Annu Rev Immunol (2009) 2.97
ATM and Artemis promote homologous recombination of radiation-induced DNA double-strand breaks in G2. EMBO J (2009) 2.94
53BP1-dependent robust localized KAP-1 phosphorylation is essential for heterochromatic DNA double-strand break repair. Nat Cell Biol (2010) 2.88
Structural and functional analysis of the middle segment of hsp90: implications for ATP hydrolysis and client protein and cochaperone interactions. Mol Cell (2003) 2.83
Structure of an Hsp90-Cdc37-Cdk4 complex. Mol Cell (2006) 2.62
Adaptor protein 3-dependent microtubule-mediated movement of lytic granules to the immunological synapse. Nat Immunol (2003) 2.56
A double-strand break repair defect in ATM-deficient cells contributes to radiosensitivity. Cancer Res (2004) 2.55
DNA-PK autophosphorylation facilitates Artemis endonuclease activity. EMBO J (2006) 2.48
Factors determining DNA double-strand break repair pathway choice in G2 phase. EMBO J (2011) 2.46
Three-dimensional structure of the human DNA-PKcs/Ku70/Ku80 complex assembled on DNA and its implications for DNA DSB repair. Mol Cell (2006) 2.43
The Mechanism of Hsp90 regulation by the protein kinase-specific cochaperone p50(cdc37). Cell (2004) 2.36
Transplantation of hematopoietic stem cells and long-term survival for primary immunodeficiencies in Europe: entering a new century, do we do better? J Allergy Clin Immunol (2010) 2.35
Structural basis for recruitment of glycogen synthase kinase 3beta to the axin-APC scaffold complex. EMBO J (2003) 2.33
Regulation of Hsp90 ATPase activity by the co-chaperone Cdc37p/p50cdc37. J Biol Chem (2002) 2.32
Structural basis for uracil recognition by archaeal family B DNA polymerases. Nat Struct Biol (2002) 2.27
Identification of a DNA nonhomologous end-joining complex in bacteria. Science (2002) 2.27
Clinical features and outcome of patients with IRAK-4 and MyD88 deficiency. Medicine (Baltimore) (2010) 2.26
Structural basis for recruitment of translesion DNA polymerase Pol IV/DinB to the beta-clamp. EMBO J (2003) 2.25
Swe1Wee1-dependent tyrosine phosphorylation of Hsp90 regulates distinct facets of chaperone function. Mol Cell (2010) 2.24
Regulation of mitotic entry by microcephalin and its overlap with ATR signalling. Nat Cell Biol (2006) 2.20
Identification of in vitro and in vivo phosphorylation sites in the catalytic subunit of the DNA-dependent protein kinase. Biochem J (2002) 2.18
Human memory B cells originate from three distinct germinal center-dependent and -independent maturation pathways. Blood (2011) 2.15
Activation segment dimerization: a mechanism for kinase autophosphorylation of non-consensus sites. EMBO J (2008) 2.12
Successful reconstitution of immunity in ADA-SCID by stem cell gene therapy following cessation of PEG-ADA and use of mild preconditioning. Mol Ther (2006) 2.08
Seckel syndrome exhibits cellular features demonstrating defects in the ATR-signalling pathway. Hum Mol Genet (2004) 1.95
Nbs1 is required for ATR-dependent phosphorylation events. EMBO J (2004) 1.95
Threonine 22 phosphorylation attenuates Hsp90 interaction with cochaperones and affects its chaperone activity. Mol Cell (2011) 1.94
Phosphoproteomic analysis reveals that PP4 dephosphorylates KAP-1 impacting the DNA damage response. EMBO J (2012) 1.91
Molecular recognition of transcriptional repressor motifs by the WD domain of the Groucho/TLE corepressor. Mol Cell (2006) 1.91
ATP-competitive inhibitors block protein kinase recruitment to the Hsp90-Cdc37 system. Nat Chem Biol (2013) 1.90
Potential role for 53BP1 in DNA end-joining repair through direct interaction with DNA. J Biol Chem (2003) 1.83
2013 IDSA clinical practice guideline for vaccination of the immunocompromised host. Clin Infect Dis (2014) 1.81
Long-term persistence of a polyclonal T cell repertoire after gene therapy for X-linked severe combined immunodeficiency. Sci Transl Med (2011) 1.81
Hematopoietic stem cell gene therapy for adenosine deaminase-deficient severe combined immunodeficiency leads to long-term immunological recovery and metabolic correction. Sci Transl Med (2011) 1.81
Outcome of hematopoietic stem cell transplantation for adenosine deaminase-deficient severe combined immunodeficiency. Blood (2012) 1.80
Trans-activation of the DNA-damage signalling protein kinase Chk2 by T-loop exchange. EMBO J (2006) 1.77
Mutations in ORC1, encoding the largest subunit of the origin recognition complex, cause microcephalic primordial dwarfism resembling Meier-Gorlin syndrome. Nat Genet (2011) 1.76
Ionizing radiation induces ataxia telangiectasia mutated kinase (ATM)-mediated phosphorylation of LKB1/STK11 at Thr-366. Biochem J (2002) 1.74
Co-chaperone regulation of conformational switching in the Hsp90 ATPase cycle. J Biol Chem (2004) 1.71
2013 IDSA clinical practice guideline for vaccination of the immunocompromised host. Clin Infect Dis (2013) 1.69
4,5-diarylisoxazole Hsp90 chaperone inhibitors: potential therapeutic agents for the treatment of cancer. J Med Chem (2007) 1.67
53BP1-mediated DNA double strand break repair: insert bad pun here. DNA Repair (Amst) (2011) 1.67
Structure of the Ire1 autophosphorylation complex and implications for the unfolded protein response. EMBO J (2011) 1.65
The impact of heterochromatin on DSB repair. Biochem Soc Trans (2009) 1.65
Healing the wounds inflicted by sleeping beauty transposition by double-strand break repair in mammalian somatic cells. Mol Cell (2004) 1.64
Structural basis for recruitment of the ATPase activator Aha1 to the Hsp90 chaperone machinery. EMBO J (2004) 1.63
Three-dimensional structure and regulation of the DNA-dependent protein kinase catalytic subunit (DNA-PKcs). Structure (2005) 1.62
A two-hybrid screen of the yeast proteome for Hsp90 interactors uncovers a novel Hsp90 chaperone requirement in the activity of a stress-activated mitogen-activated protein kinase, Slt2p (Mpk1p). Eukaryot Cell (2005) 1.62
Neonatal diagnosis of severe combined immunodeficiency leads to significantly improved survival outcome: the case for newborn screening. Blood (2011) 1.62
Structure and functional relationships of Hsp90. Curr Cancer Drug Targets (2003) 1.59
Chromatin architecture and the generation of antigen receptor diversity. Cell (2009) 1.59
Hsp90-dependent activation of protein kinases is regulated by chaperone-targeted dephosphorylation of Cdc37. Mol Cell (2008) 1.57
ATM mediates phosphorylation at multiple p53 sites, including Ser(46), in response to ionizing radiation. J Biol Chem (2002) 1.56
Insights into histone code syntax from structural and biochemical studies of CARM1 methyltransferase. EMBO J (2007) 1.55
Regulation of DNA replication through Sld3-Dpb11 interaction is conserved from yeast to humans. Curr Biol (2011) 1.54
Impact of DNA ligase IV on the fidelity of end joining in human cells. Nucleic Acids Res (2003) 1.52
The ATPase-dependent chaperoning activity of Hsp90a regulates thick filament formation and integration during skeletal muscle myofibrillogenesis. Development (2008) 1.52
Structural model of full-length human Ku70-Ku80 heterodimer and its recognition of DNA and DNA-PKcs. EMBO Rep (2006) 1.50
Structural basis for recruitment of BRCA2 by PALB2. EMBO Rep (2009) 1.50
Structure and specificity of the vertebrate anti-mutator uracil-DNA glycosylase SMUG1. Mol Cell (2003) 1.49
Identification of the Axin and Frat binding region of glycogen synthase kinase-3. J Biol Chem (2001) 1.49
Structural and mechanistic insights into ras association domains of phospholipase C epsilon. Mol Cell (2006) 1.48
Crystal structure of alanine:glyoxylate aminotransferase and the relationship between genotype and enzymatic phenotype in primary hyperoxaluria type 1. J Mol Biol (2003) 1.47
53BP1 promotes ATM activity through direct interactions with the MRN complex. EMBO J (2009) 1.47
Activation segment exchange: a common mechanism of kinase autophosphorylation? Trends Biochem Sci (2007) 1.45
XLF-Cernunnos promotes DNA ligase IV-XRCC4 re-adenylation following ligation. Nucleic Acids Res (2008) 1.44
HAE international home therapy consensus document. Allergy Asthma Clin Immunol (2010) 1.42
High-throughput screening assay for inhibitors of heat-shock protein 90 ATPase activity. Anal Biochem (2004) 1.42
Mutations in STAT3 and diagnostic guidelines for hyper-IgE syndrome. J Allergy Clin Immunol (2010) 1.36
Sensitization to radiation and alkylating agents by inhibitors of poly(ADP-ribose) polymerase is enhanced in cells deficient in DNA double-strand break repair. Mol Cancer Ther (2010) 1.34
Analysis of DNA ligase IV mutations found in LIG4 syndrome patients: the impact of two linked polymorphisms. Hum Mol Genet (2004) 1.33
Nbs1 promotes ATM dependent phosphorylation events including those required for G1/S arrest. Oncogene (2002) 1.33
Visualization of DNA-induced conformational changes in the DNA repair kinase DNA-PKcs. EMBO J (2003) 1.32
Crystal structure of the rad9-rad1-hus1 DNA damage checkpoint complex--implications for clamp loading and regulation. Mol Cell (2009) 1.31