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Fanconi Anemia Complementation Group Proteins (8):
- Fanconi Anemia Complementation Group G Protein
- BRCA2 Protein
- Fanconi Anemia Complementation Group C Protein
- Fanconi Anemia Complementation Group F Protein
- Fanconi Anemia Complementation Group D2 Protein
- Fanconi Anemia Complementation Group E Protein
- Fanconi Anemia Complementation Group L Protein
- Fanconi Anemia Complementation Group A Protein
Recent article
Fanconi Anemia Complementation Group Proteins
Abstract
We characterize a previously unrecognized nuclease, Fanconi anemia-associated nuclease 1 (FAN1), that promotes ICL repair in a manner strictly dependent on its ability to accumulate at or near sites of DNA damage and that relies on mono-ubiquitylation of the ID complex. Thus, the mono-ubiquitylated...
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PMID: 20671156
PDF is available here.
PDF is available here.
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Abstract
We address the role of FANCJ and MLH1 in DNA crosslink processing and how their functions could be linked in checkpoint and/or recombination pathways. We speculate that after DNA crosslink processing and repair, the FANCJ/MLH1 interaction is critical for recovery and restart of replication. These id...
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PMID: 20658644
PDF is available here.
PDF is available here.
Abstract
We have discovered that BRCA1 binding to FANCJ regulates DNA damage repair choice. Thus, when FANCJ binding to BRCA1 is ablated, the molecular mechanism chosen for the repair of damaged DNA is dramatically altered. Specifically, a FANCJ protein that cannot be phosphorylated at serine 990 or bind BRC...
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PMID: 20173781
PDF is available here.
PDF is available here.
Abstract
We have characterized the signal transduction pathways regulating the catabolisis of p45/NF-E2, a bZIP factor activating the erythroid and megakaryocytic gene transcription. Through use of different approaches including nano-scale proteomics, we show that activated-JNK, or Phospho-JNK (P-JNK), physi...
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PMID: 19966288
PDF is available here.
PDF is available here.
Abstract
Fanconi anemia (FA) is a recessive genetic disorder characterized by developmental defects, bone marrow failure, and cancer susceptibility. The complete set of FA genes has only been identified recently and seems to be uniquely conserved among vertebrates. Fanconi anemia proteins have been implicate...
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PMID: 20515746
PDF is available here.
PDF is available here.
Abstract
We showed that FANCI-FANCD2 is required for replication-coupled ICL repair in S phase. Removal of FANCD2 from extracts inhibits both nucleolytic incisions near the ICL and translesion DNA synthesis past the lesion. Reversal of these defects requires ubiquitylated FANCI-FANCD2. Our results show that...
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PMID: 19965384
PDF is available here.
PDF is available here.
Abstract
Iron, in the form of ferric nitrilotriacetate, increased oxidative stress and up-regulated HMOX1 gene expression. Iron did not affect mRNA or protein levels of Bach1, a repressor of HMOX1. Silencing the up-regulation of HMOX1 nuclear factor-erythroid 2-related factor 2 (Nrf2) by Nrf2-siRNA decreased...
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PMID: 19777608
PDF is available here.
PDF is available here.
Molecular Cell (35)5 2009
Abstract
We developed a chromatin-IP-based strategy termed eChIP and detected association of multiple FA proteins with DNA crosslinks in vivo. Interdependence analyses revealed that crosslink-specific enrichment of various FA proteins is controlled by distinct mechanisms. BRCA-related FA proteins (BRCA2, FAN...
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PMID: 19748364
PDF is available here.
PDF is available here.
Mutation Research (668)1-2 2009
Abstract
We consider potential sources of endogenous DNA damage, or endogenous stresses, to which FA proteins may respond. These include ICLs formed by products of lipid peroxidation, and other forms of oxidative DNA damage. FA proteins may also potentially respond to telomere shortening or replication stres...
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PMID: 19774700
PDF is available here.
PDF is available here.
Mutation Research (668)1-2 2009
Abstract
We present cDNA sequences, genetic mapping, and genomic analyses for the four previously undescribed zebrafish FA genes (fanci, fancj, fancm, and fancn), and show that they reverted to single copy after the teleost genome duplication. We tested the hypothesis that FA genes are expressed during embry...
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PMID: 19101574
PDF is available here.
PDF is available here.
Mutation Research (668)1-2 2009
Abstract
We focus on the discovery, structure, and function of the FANCM-FAAP24 and FANCJ proteins....
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PMID: 19379763
PDF is available here.
PDF is available here.
Mutation Research (668)1-2 2009
Abstract
We have attempted to reconcile and integrate numerous observations into a model in which FANC proteins coordinate the following physiological events during DNA crosslink repair: (a) activating a FANCM-ATR-dependent S-phase checkpoint, (b) mediating enzymatic replication-fork breakage and crosslink u...
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PMID: 19622404
PDF is available here.
PDF is available here.
Rinsho Ketsueki (50)7 2009
Abstract
Journal of Biological Chemistry (284)27 2009
Abstract
We have examined the ability of FANCJ to unwind DNA molecules with specific base damage that can be mutagenic or lethal. FANCJ was inhibited by a single thymine glycol, but not 8-oxoguanine, in either the translocating or nontranslocating strands of the helicase substrate. In contrast, the human Rec...
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PMID: 19419957
PDF is available here.
PDF is available here.
Abstract
Abstract
The FANCJ family of DNA helicases is emerging as an important group of proteins for the prevention of human disease, cancer, and chromosomal instability. FANCJ was identified by its association with breast cancer, and is implicated in Fanconi Anemia. Proteins with sequence similarity to FANCJ are im...
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PMID: 19099189
PDF is available here.
PDF is available here.
DNA Repair (8)4 2009
Abstract
We review and extract the key features of the enzymatic cascade required for the monoubiquitylation of the FANCD2/FANCI complex and attempt to include recent findings into a coherent mechanism. As this part of the FA pathway is still far from fully understood, we raise several points that must be ad...
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PMID: 19264559
PDF is available here.
PDF is available here.
Abstract
We will review current research directed at identifying FA genes and understanding the function of FA proteins in DNA damage responses. We will also examine interactions of FA proteins with other repair proteins and pathways, including signaling networks, which are potentially involved in ICL repair...
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PMID: 19200054
PDF is available here.
PDF is available here.
Neuropsychopharmacology (34)1 2009
Abstract
Abstract
We begin this review with a summary of the strategies employed by prokaryotes and eukaryotes to tolerate obstacles to the progression of replication forks. We then summarize what we know about Fanconi anemia with an emphasis on biochemical aspects, and discuss how the Fanconi anemia network, a late...
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PMID: 19728769
PDF is available here.
PDF is available here.
Molecular Cell (32)6 2008
Abstract
We minimally reconstitute this monoubiquitination reaction with Ube2t and the FANCL protein, revealing that monoubiquitination is stimulated by a conserved RWD-like domain in FANCL. Furthermore, addition of the FANCI protein enhances monoubiquitination and also restricts it to the in vivo substrate...
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PMID: 19111657
PDF is available here.
PDF is available here.
Abstract
We discuss the functional link between FA proteins and oxidative DNA damage response/repair, with emphasis on the implication of oxidative stress in the pathophysiology and abnormal hematopoiesis in FA....
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PMID: 18627348
PDF is available here.
PDF is available here.
Abstract
This finding opens a new view of the pathophysiology of emphysema and could provide the basis for new therapeutic approaches based on preservation and/or restoration of such equilibrium....
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PMID: 18559366
PDF is available here.
PDF is available here.
Biochemistry (Washington) (47)37 2008
Abstract
We show that the recognition mechanism is complex and best modeled by two equilibrium conformations of BRCA1-BRCT in the free state that both interact with a phosphopeptide, with dissociation constants ( K d) in the micromolar range. We show that the apparent global dissociation constant derived fro...
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PMID: 18717574
PDF is available here.
PDF is available here.
Abstract
Abstract
We associate the FA proteins with the Notch1 developmental pathway through a direct protein-protein interaction between the FA core complex and the hairy enhancer of split 1 (HES1). HES1 interaction with FA core complex members is dependent on a functional FA pathway. Cells depleted of HES1 exhibit...
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PMID: 18550849
PDF is available here.
PDF is available here.
Abstract
We report here that multiple FA proteins were downregulated during the proliferative arrest of primary human keratinocytes and HeLa cells, and that the observed regulation was at a transcriptional level. Proliferative stimuli such as expression of HPV16 E7 as well as E2F1 overexpression in primary c...
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PMID: 18438432
PDF is available here.
PDF is available here.
Current Biology (18)14 2008
Abstract
G-rich regions have the potential to form G4 DNA upon replication, which can lead to genomic instability. FANCJ, a G4 DNA helicase, has been shown to be critical for the stability of regions that match the G4 signature motif by experiments analyzing its nematode homolog.
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PMID: 18644339
PDF is available here.
PDF is available here.
Abstract
We aimed to unravel the transcriptional control of Brip1 and to determine its expression levels in a set of 101 primary invasive breast carcinomas. Transcription of Brip1 was found to be cell growth-related and controlled by the E2F/retinoblastoma (Rb) pathway through a conserved E2F-responsive site...
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PMID: 18345034
PDF is available here.
PDF is available here.
Current Biology (18)12 2008
Abstract
We demonstrate that G4 DNA-a sequence motif that folds into quadruplex structures in vitro [4, 5]-is highly mutagenic in vivo and is removed from genomes that lack dog-1, the C. elegans ortholog of mammalian FANCJ [6, 7], which is mutated in Fanconi anemia patients [8-11]. We show that sequences tha...
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PMID: 18538569
PDF is available here.
PDF is available here.
Abstract
Human Molecular Genetics (17)11 2008
Abstract
We have shown previously that the FANCM constituent of the complex contains a highly conserved helicase domain and an associated ATP-dependent DNA translocase activity. Here we show that FANCM also possesses an ATP-independent binding activity and an ATP-dependent bi-directional branch-point translo...
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PMID: 18285517
PDF is available here.
PDF is available here.
Abstract
FANCJ mutations are associated with breast cancer and genetically linked to the bone marrow disease Fanconi anemia (FA). The genomic instability of FA-J mutant cells suggests that FANCJ helicase functions in the replicational stress response. A putative helicase with sequence similarity to FANCJ in...
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PMID: 18426915
PDF is available here.
PDF is available here.
Abstract
We examined the effect of FANCM expression on the integrity and localization of the FA core complex. FANCM was exclusively localized to chromatin fractions and underwent cell cycle-dependent phosphorylation and dephosphorylation. FANCM-depleted HeLa cells had an intact FA core complex but were defec...
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PMID: 18174376
PDF is available here.
PDF is available here.
Haematologica (93)4 2008
Abstract
Findings from the German Fanconi Anemia Registry cohort validate prior risk estimates, and strongly support the concept that Fanconi anemia is a highly penetrant cancer susceptibility syndrome with early onset of acute myeloid leukemia and slightly later onset of specific solid tumors....
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PMID: 18322251
PDF is available here.
PDF is available here.
Haematologica (93)4 2008
Abstract
Abstract
We show that DOG-1 is the Caenorhabditis elegans homologue of FANCJ, a helicase mutated in FA-J patients. DOG-1 performs a conserved role in ICL repair, as dog-1 mutants are hypersensitive to ICL-inducing agents, but not to UVC irradiation or X rays. Genetic analysis indicated that dog-1 is epistati...
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PMID: 18086896
PDF is available here.
PDF is available here.
Biochimica et Biophysica Acta (1779)3 2008
Abstract
We investigated the effects of ZnMP on Bach1 mRNA and protein levels in human hepatoma Huh-7 cells by quantitative RT-PCR and Western blots. We found that ZnMP markedly up-regulated HO-1 mRNA and protein levels, and rapidly and significantly decreased Bach1 protein levels by increasing degradation o...
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PMID: 18325350
PDF is available here.
PDF is available here.
Abstract
We sought to evaluate the contribution of FANCJ gene alterations regarding breast cancer susceptibility among our cohort of 96 breast cancer individuals from high-risk non-BRCA1/2 French Canadian families. No deleterious mutation, exon deletion, or retention of intronic portions could be identified....
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PMID: 18414782
PDF is available here.
PDF is available here.
Human Mutation (29)1 2008
Abstract
We report a comprehensive genetic subtyping approach for FA that is primarily based on mutation screening, supplemented by protein expression analysis and by functional assays to test for pathogenicity of unclassified variants. Of 80 FA cases analyzed, 73 (91%) were successfully subtyped. In total,...
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PMID: 17924555
PDF is available here.
PDF is available here.
Cellular Oncology (30)4 2008
Abstract
MS-MLPA showed promoter methylation of FANCC in one AML and three ALL samples, while FANCL was found methylated in one ALL sample. Bisulphite sequencing of promoter regions confirmed hypermethylation in all cases. In addition, samples with hypermethylation of either FANCC or FANCL appeared more sens...
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PMID: 18607065
PDF is available here.
PDF is available here.
Abstract
We identified proteins likely associated to the molecular signaling pathways involved in DNA repair of interstrand cross-link lesions and in mechanisms of genomic stability mediated by FA/BRCA pathways. We compared protein maps resolved by bidimensional electrophoresis and analyzed differentially ex...
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PMID: 17977515
PDF is available here.
PDF is available here.
Abstract
We report that the miR-K12-11 miRNA encoded by Kaposi's-sarcoma-associated herpes virus (KSHV) shows significant homology to cellular miR-155, including the entire miRNA 'seed' region. Using a range of assays, we show that expression of physiological levels of miR-K12-11 or miR-155 results in the do...
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PMID: 18075594
PDF is available here.
PDF is available here.
Journal of Virology (81)23 2007
Abstract
We found that KSHV-miR-K12-11 shares 100% seed sequence homology with hsa-miR-155, an miRNA frequently found to be up-regulated in lymphomas and critically important for B-cell development. Based on this seed sequence homology, we hypothesized that both miRNAs regulate a common set of target genes a...
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PMID: 17881434
PDF is available here.
PDF is available here.
Gastroenterology (133)4 2007
Abstract
Down-regulation of HCV replication using an antagomir targeted to miR-122 is effective, specific, and selective. Increasing HO-1, by silencing the Bach1 gene or by treatment with cobalt protoporphyrin or heme, decreases HCV replication. Thus, miR-122 plays an important role in the regulation of HCV...
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PMID: 17919492
PDF is available here.
PDF is available here.
Genes to Cells (12)10 2007
Abstract
We knockout the genes for Mus81 and FANCB, a component of the FA core complex, in the human Nalm-6 cell line. We show that Mus81 plays an important role in cell proliferation to suppress cell death when FANCB is missing, indicating a functional linkage between Mus81 and the FA pathway. In DNA cross-...
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PMID: 17903171
PDF is available here.
PDF is available here.
Nature Reviews: Genetics (8)10 2007
Abstract
I discuss what is known about FA proteins and their interactive network, and what remains to be discovered....
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PMID: 17768402
PDF is available here.
PDF is available here.
Abstract
We report the association of BACH1 with a distinct BRCA1/BRCA2-containing complex during the S phase of the cell cycle. Depletion of BACH1 or BRCA1 using small interfering RNAs results in delayed entry into the S phase of the cell cycle. Such timely progression through S phase requires the helicase...
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PMID: 17664283
PDF is available here.
PDF is available here.
Abstract
We investigated the potential interaction of FANCJ with replication protein A (RPA), a single-stranded DNA-binding protein implicated in both DNA replication and repair. FANCJ and RPA were shown to coimmunoprecipitate most likely through a direct interaction of FANCJ and the RPA70 subunit. Moreover,...
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PMID: 17596542
PDF is available here.
PDF is available here.
Abstract
FASEB Journal (21)11 2007
Abstract
EMBO Journal (26)13 2007
The FANCJ/MutLalpha interaction is required for correction of the cross-link response in FA-J cells.
Abstract
We show that FANCJ interacts with the mismatch repair complex MutLalpha, composed of PMS2 and MLH1. Specifically, FANCJ directly interacts with MLH1 independent of BRCA1, through its helicase domain. Genetic studies reveal that FANCJ helicase activity and MLH1 binding, but not BRCA1 binding, are ess...
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PMID: 17581638
PDF is available here.
PDF is available here.
Abstract
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. A FANCD2 protein sequence-based homology search facilitated the discovery of FANCI, a second monoubiquiti...
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PMID: 17460694
PDF is available here.
PDF is available here.
Abstract
We tested the contribution of the Fanconi anemia (FA) DNA repair pathway. TMZ and BCNU treatment of FA-proficient cell lines led to a dose- and time-dependent increase in FANCD2 mono-ubiquitination and FANCD2 nuclear foci formation, both hallmarks of FA pathway activation. The FA-deficient cells wer...
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PMID: 17221219
PDF is available here.
PDF is available here.
Abstract
We describe the FA protein FANCI, identified as an ATM/ATR kinase substrate required for resistance to mitomycin C. FANCI shares sequence similarity with FANCD2, likely evolving from a common ancestral gene. The FANCI protein associates with FANCD2 and, together, as the FANCI-FANCD2 (ID) complex, lo...
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PMID: 17412408
PDF is available here.
PDF is available here.
Abstract
From a total of 125 patients included in the Registry of Fanconi Anaemia, samples from 102 patients were available for subtyping analyses. In 89 cases the subtype could be determined and in 8 cases exclusions of common complementation groups were made. Compared with other international studies, a sk...
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PMID: 17105750
PDF is available here.
PDF is available here.
EMBO Journal (26)5 2007
Abstract
We have induced subnuclear accumulation of DNA damage to prove that histone H2AX is a novel component of the FA/BRCA pathway in response to stalled replication forks. Analyses of cells from H2AX knockout mice or expressing a nonphosphorylable H2AX (H2AX(S136A/S139A)) indicate that phosphorylated H2A...
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PMID: 17304220
PDF is available here.
PDF is available here.
Cancer Research (67)5 2007
Abstract
We assessed the growth of isogenic human cancer cells that differed only in the presence or absence of single FA genes upon treatment with 880 active drugs and 40,000 diverse compounds. We identified several compounds to which FA pathway-deficient cells were more sensitive than FA pathway-proficient...
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PMID: 17332347
PDF is available here.
PDF is available here.
Molecular Cell (25)4 2007
Abstract
FAAP24, a new XPF endonuclease family member identified by in a recent issue of Molecular Cell, heterodimerizes with FANCM, binds unwound DNA, and reveals how the Fanconi anemia core complex concentrates DNA repair proteins at stalled replication forks.
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PMID: 17317622
PDF is available here.
PDF is available here.
Molecular Cell (25)3 2007
Abstract
We describe the identification of FAAP24, a protein that targets FANCM to structures that mimic intermediates formed during the replication/repair of damaged DNA. FAAP24 shares homology with the XPF family of flap/fork endonucleases, associates with the C-terminal region of FANCM, and is a component...
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PMID: 17289582
PDF is available here.
PDF is available here.