Gene Symbol: Ercc1
Description: excision repair cross-complementing rodent repair deficiency, complementation group 1
Alias: Ercc-1, DNA excision repair protein ERCC-1
Species: mouse
Products:     Ercc1

Top Publications

  1. Niedernhofer L, Essers J, Weeda G, Beverloo B, de Wit J, Muijtjens M, et al. The structure-specific endonuclease Ercc1-Xpf is required for targeted gene replacement in embryonic stem cells. EMBO J. 2001;20:6540-9 pubmed
    The Ercc1-Xpf heterodimer, a highly conserved structure-specific endonuclease, functions in multiple DNA repair pathways that are pivotal for maintaining genome stability, including nucleotide excision repair, interstrand crosslink ..
  2. Dollé M, Busuttil R, Garcia A, Wijnhoven S, van Drunen E, Niedernhofer L, et al. Increased genomic instability is not a prerequisite for shortened lifespan in DNA repair deficient mice. Mutat Res. 2006;596:22-35 pubmed
    ..a consequence of NER deficiency, we crossed four different mouse mutants, Xpa-/-, Ercc6(Csb)-/-, Ercc2(Xpd)m/m and Ercc1-/m, with mice harboring lacZ-reporter genes to assess mutant frequencies and spectra in different organs during ..
  3. Doig J, Anderson C, Lawrence N, Selfridge J, Brownstein D, Melton D. Mice with skin-specific DNA repair gene (Ercc1) inactivation are hypersensitive to ultraviolet irradiation-induced skin cancer and show more rapid actinic progression. Oncogene. 2006;25:6229-38 pubmed
    b>Ercc1 has an essential role in the nucleotide excision repair (NER) pathway that protects against ultraviolet (UV)-induced DNA damage and is also involved in additional repair pathways...
  4. Niedernhofer L, Garinis G, Raams A, Lalai A, Robinson A, Appeldoorn E, et al. A new progeroid syndrome reveals that genotoxic stress suppresses the somatotroph axis. Nature. 2006;444:1038-43 pubmed
    XPF-ERCC1 endonuclease is required for repair of helix-distorting DNA lesions and cytotoxic DNA interstrand crosslinks. Mild mutations in XPF cause the cancer-prone syndrome xeroderma pigmentosum...
  5. Lawrence N, Sacco J, Brownstein D, Gillingwater T, Melton D. A neurological phenotype in mice with DNA repair gene Ercc1 deficiency. DNA Repair (Amst). 2008;7:281-91 pubmed publisher
    ..b>Ercc1 is essential for nucleotide excision repair and is also involved in recombination repair and the repair of ..
  6. Niedernhofer L, Odijk H, Budzowska M, van Drunen E, Maas A, Theil A, et al. The structure-specific endonuclease Ercc1-Xpf is required to resolve DNA interstrand cross-link-induced double-strand breaks. Mol Cell Biol. 2004;24:5776-87 pubmed
    ..The mechanism of ICL repair in mammalian cells is poorly understood. However, genetic data implicate the Ercc1-Xpf endonuclease and proteins required for homologous recombination-mediated double-strand break (DSB) repair...
  7. Selfridge J, Hsia K, Redhead N, Melton D. Correction of liver dysfunction in DNA repair-deficient mice with an ERCC1 transgene. Nucleic Acids Res. 2001;29:4541-50 pubmed
    The ERCC1 gene is essential for the repair of UV-induced DNA damage. Unlike most genes in the nucleotide excision repair (NER) pathway, ERCC1 is also involved in recombinational repair...
  8. Bhagwat N, Olsen A, Wang A, Hanada K, Stuckert P, Kanaar R, et al. XPF-ERCC1 participates in the Fanconi anemia pathway of cross-link repair. Mol Cell Biol. 2009;29:6427-37 pubmed publisher
    ..required for homologous recombination, translesion synthesis, and at least two endonucleases, MUS81-EME1 and XPF-ERCC1. ICLs cause replication-dependent DNA double-strand breaks (DSBs), and MUS81-EME1 facilitates DSB formation...
  9. de Waard M, van der Pluijm I, Zuiderveen Borgesius N, Comley L, Haasdijk E, Rijksen Y, et al. Age-related motor neuron degeneration in DNA repair-deficient Ercc1 mice. Acta Neuropathol. 2010;120:461-75 pubmed publisher
    ..Here, we analyzed neurodegenerative abnormalities in the spinal cord of progeroid Ercc1(Delta/-) mice that are impaired in several DNA repair systems, i.e...

More Information


  1. Chen Q, Liu K, Robinson A, Clauson C, Blair H, Robbins P, et al. DNA damage drives accelerated bone aging via an NF-?B-dependent mechanism. J Bone Miner Res. 2013;28:1214-28 pubmed publisher
    ..Excision repair cross complementary group 1-xeroderma pigmentosum group F (ERCC1-XPF) is an evolutionarily conserved structure-specific endonuclease that is required for multiple DNA repair ..
  2. de Boer J, Hoeijmakers J. Cancer from the outside, aging from the inside: mouse models to study the consequences of defective nucleotide excision repair. Biochimie. 1999;81:127-37 pubmed the paradoxical absence of cancer development in TTD and CS despite their NER deficiencies, and the role of the ERCC1 gene in mitotic recombination and cross-link repair...
  3. Hsia K, Millar M, King S, Selfridge J, Redhead N, Melton D, et al. DNA repair gene Ercc1 is essential for normal spermatogenesis and oogenesis and for functional integrity of germ cell DNA in the mouse. Development. 2003;130:369-78 pubmed
    b>Ercc1 is essential for nucleotide excision repair (NER) but, unlike other NER proteins, Ercc1 and Xpf are also involved in recombination repair pathways...
  4. Winter A, Samuel K, Hsia K, Melton D. The repair and recombination enzyme ERCC1 is not required for immunoglobulin class switching. DNA Repair (Amst). 2003;2:561-9 pubmed
    ..It has previously been suggested that the nucleotide excision repair protein ERCC1 may be involved in CSR due to its known roles in removal of 3' single-stranded tails in various types of ..
  5. Zhu X, Niedernhofer L, Kuster B, Mann M, Hoeijmakers J, de Lange T. ERCC1/XPF removes the 3' overhang from uncapped telomeres and represses formation of telomeric DNA-containing double minute chromosomes. Mol Cell. 2003;12:1489-98 pubmed
    ..Here we report that ERCC1/XPF-deficient cells retained the telomeric overhang after TRF2 inhibition, identifying this nucleotide excision ..
  6. Crossan G, van der Weyden L, Rosado I, Langevin F, Gaillard P, McIntyre R, et al. Disruption of mouse Slx4, a regulator of structure-specific nucleases, phenocopies Fanconi anemia. Nat Genet. 2011;43:147-52 pubmed publisher
    ..a crucial requirement for Btbd12 (also known as Slx4) to interact with the structure-specific endonuclease Xpf-Ercc1 to promote crosslink repair...
  7. McWhir J, Selfridge J, Harrison D, Squires S, Melton D. Mice with DNA repair gene (ERCC-1) deficiency have elevated levels of p53, liver nuclear abnormalities and die before weaning. Nat Genet. 1993;5:217-24 pubmed
    ..Elevated p53 levels were detected in liver, brain and kidney, supporting the hypothesised role for p53 as a monitor of DNA damage. ..
  8. Tilstra J, Robinson A, Wang J, Gregg S, Clauson C, Reay D, et al. NF-?B inhibition delays DNA damage-induced senescence and aging in mice. J Clin Invest. 2012;122:2601-12 pubmed publisher
    ..IKK/NF-?B inhibitors are sufficient to attenuate this damage and could provide clinical benefit for degenerative changes associated with accelerated aging disorders and normal aging. ..
  9. Prasher J, Lalai A, Heijmans Antonissen C, Ploemacher R, Hoeijmakers J, Touw I, et al. Reduced hematopoietic reserves in DNA interstrand crosslink repair-deficient Ercc1-/- mice. EMBO J. 2005;24:861-71 pubmed
    The ERCC1-XPF heterodimer is a structure-specific endonuclease involved in both nucleotide excision repair and interstrand crosslink repair...
  10. Ahmad A, Robinson A, Duensing A, van Drunen E, Beverloo H, Weisberg D, et al. ERCC1-XPF endonuclease facilitates DNA double-strand break repair. Mol Cell Biol. 2008;28:5082-92 pubmed publisher
    b>ERCC1-XPF endonuclease is required for nucleotide excision repair (NER) of helix-distorting DNA lesions...
  11. Weeda G, Donker I, de Wit J, Morreau H, Janssens R, Vissers C, et al. Disruption of mouse ERCC1 results in a novel repair syndrome with growth failure, nuclear abnormalities and senescence. Curr Biol. 1997;7:427-39 pubmed
    The structure-specific ERCC1/XPF endonuclease complex that contains the ERCC1 and XPF subunits is implicated in the repair of two distinct types of lesions in DNA: nucleotide excision repair (NER) for ultraviolet-induced lesions and ..
  12. Sepe S, Milanese C, Gabriels S, Derks K, Payan Gomez C, van Ijcken W, et al. Inefficient DNA Repair Is an Aging-Related Modifier of Parkinson's Disease. Cell Rep. 2016;15:1866-75 pubmed publisher
    ..that dermal fibroblasts from PD patients display flawed nucleotide excision repair (NER) capacity and that Ercc1 mutant mice with mildly compromised NER exhibit typical PD-like pathological alterations, including decreased ..
  13. Mohrenweiser H, Carrano A, Fertitta A, Perry B, Thompson L, Tucker J, et al. Refined mapping of the three DNA repair genes, ERCC1, ERCC2, and XRCC1, on human chromosome 19. Cytogenet Cell Genet. 1989;52:11-4 pubmed
    Three DNA repair genes, ERCC1, ERCC2, and XRCC1, have been regionally mapped on human chromosome 19. ERCC2 and XRCC1 have been assigned to bands q13.2----q13.3 by in situ hybridization using fluorescently-labeled cosmid probes...
  14. Hajkova P, Jeffries S, Lee C, Miller N, Jackson S, Surani M. Genome-wide reprogramming in the mouse germ line entails the base excision repair pathway. Science. 2010;329:78-82 pubmed publisher
    ..We demonstrate that DNA repair through BER represents a core component of genome-wide DNA demethylation in vivo and provides a mechanistic link to the extensive chromatin remodeling in developing PGCs. ..
  15. Kirschner K, Singh R, Prost S, Melton D. Characterisation of Ercc1 deficiency in the liver and in conditional Ercc1-deficient primary hepatocytes in vitro. DNA Repair (Amst). 2007;6:304-16 pubmed
    The ERCC1/XPF complex is responsible for incision at the 5' side of the lesion during nucleotide excision repair and is also involved in homologous recombination and interstrand cross-link repair...
  16. Hagelstrom R, Blagoev K, Niedernhofer L, Goodwin E, Bailey S. Hyper telomere recombination accelerates replicative senescence and may promote premature aging. Proc Natl Acad Sci U S A. 2010;107:15768-73 pubmed publisher
    ..Similarly, XFE progeroid syndrome results from defects in the ERCC1-XPF DNA repair endonuclease...
  17. Karakasilioti I, Kamileri I, Chatzinikolaou G, Kosteas T, Vergadi E, Robinson A, et al. DNA damage triggers a chronic autoinflammatory response, leading to fat depletion in NER progeria. Cell Metab. 2013;18:403-15 pubmed publisher
    ..However, the underlying mechanisms remain poorly understood. Using mice carrying an ERCC1-XPF DNA repair defect systematically or in adipocytes, we show that DNA damage signaling triggers a chronic ..
  18. Chatzinikolaou G, Apostolou Z, Aid Pavlidis T, Ioannidou A, Karakasilioti I, Papadopoulos G, et al. ERCC1-XPF cooperates with CTCF and cohesin to facilitate the developmental silencing of imprinted genes. Nat Cell Biol. 2017;19:421-432 pubmed publisher
    ..tagging approach in mice, we show that the nucleotide excision repair (NER) structure-specific endonuclease ERCC1-XPF complex interacts with the insulator binding protein CTCF, the cohesin subunits SMC1A and SMC3 and with MBD2; ..
  19. Griffin C, Waard H, Deans B, Thacker J. The involvement of key DNA repair pathways in the formation of chromosome rearrangements in embryonic stem cells. DNA Repair (Amst). 2005;4:1019-27 pubmed
    ..We chose cells deficient in Ku70 (DNA end joining), Xrcc2 (gene conversion), Ercc1 (single-strand annealing) and Csb (transcription-coupled repair) to represent potentially-important DNA repair ..
  20. Alemdehy M, Haanstra J, de Looper H, van Strien P, Verhagen Oldenampsen J, Caljouw Y, et al. ICL-induced miR139-3p and miR199a-3p have opposite roles in hematopoietic cell expansion and leukemic transformation. Blood. 2015;125:3937-48 pubmed publisher
    ..The molecular mechanisms responsible for these defects are largely unknown. Using Ercc1-deficient mice, we show that Trp53 is responsible for ICL-induced bone marrow failure and that loss of Trp53 is ..
  21. Cho J, Kook S, Robinson A, Niedernhofer L, Lee B. Cell autonomous and nonautonomous mechanisms drive hematopoietic stem/progenitor cell loss in the absence of DNA repair. Stem Cells. 2013;31:511-25 pubmed publisher
    ..Herein, we demonstrate that mice expressing reduced levels of ERCC1-XPF DNA repair endonuclease (Ercc1-/? mice) spontaneously display a progressive decline in the number and function ..
  22. Dollé M, Kuiper R, Roodbergen M, Robinson J, de Vlugt S, Wijnhoven S, et al. Broad segmental progeroid changes in short-lived Ercc1(-/?7) mice. Pathobiol Aging Age Relat Dis. 2011;1: pubmed publisher
    ..The ERCC1 protein is involved in three genome maintenance systems: nucleotide excision repair, interstrand cross-link repair,..
  23. Nunez F, Chipchase M, Clarke A, Melton D. Nucleotide excision repair gene (ERCC1) deficiency causes G(2) arrest in hepatocytes and a reduction in liver binucleation: the role of p53 and p21. FASEB J. 2000;14:1073-82 pubmed
    ..The ERCC1 protein is essential for NER, but ERCC1 knockout mice are not a model for XP...
  24. van de Ven M, Andressoo J, Holcomb V, von Lindern M, Jong W, De Zeeuw C, et al. Adaptive stress response in segmental progeria resembles long-lived dwarfism and calorie restriction in mice. PLoS Genet. 2006;2:e192 pubmed
    ..Specific (but not all) types of genome instability may thus engage a conserved response to stress that evolved to cope with environmental pressures such as food shortage. ..
  25. Flores R, Clauson C, Cho J, Lee B, McGowan S, Baker D, et al. Expansion of myeloid-derived suppressor cells with aging in the bone marrow of mice through a NF-?B-dependent mechanism. Aging Cell. 2017;16:480-487 pubmed publisher
    ..natural aged mice and in a mouse model of a human progeroid syndrome caused by defective repair of DNA damage (ERCC1-deficient mice)...
  26. Song L, Ritchie A, McNeil E, Li W, Melton D. Identification of DNA repair gene Ercc1 as a novel target in melanoma. Pigment Cell Melanoma Res. 2011;24:966-71 pubmed publisher
    ..The DNA repair protein, ERCC1, is needed to remove cisplatin-induced DNA damage...
  27. Van Sloun P, Jansen J, Weeda G, Mullenders L, van Zeeland A, Lohman P, et al. The role of nucleotide excision repair in protecting embryonic stem cells from genotoxic effects of UV-induced DNA damage. Nucleic Acids Res. 1999;27:3276-82 pubmed
    ..Cytotoxic and mutagenic effects of photolesions were determined in ES cells differing in NER activity. ERCC1-deficient ES cells were hypermutable (10-fold) compared to wild-type cells, indicating that at physiologically ..
  28. van Beek A, Hugenholtz F, Meijer B, Sovran B, Perdijk O, Vermeij W, et al. Frontline Science: Tryptophan restriction arrests B cell development and enhances microbial diversity in WT and prematurely aging Ercc1-/?7 mice. J Leukoc Biol. 2017;101:811-821 pubmed publisher
    ..restriction in young wild-type (WT) mice (118-wk life span) and in DNA-repair deficient, premature-aged (Ercc1-/?7 ) mice (20-wk life span)...
  29. Song L, Winter A, Selfridge J, Melton D. A novel transcript for DNA repair gene Ercc1 in mouse skin. Transgenic Res. 2011;20:109-22 pubmed publisher UV is the skin and we have investigated the possibility that expression of the nucleotide excision repair gene, Ercc1, may display different properties in the skin to deal with a more demanding role in that tissue...
  30. Smith S, Robinson A, Niedernhofer L, Hetman M. Downregulation of cholesterol biosynthesis genes in the forebrain of ERCC1-deficient mice. Neurobiol Dis. 2012;45:1136-44 pubmed publisher
    ..including deficiency of the Excision Repair Cross-Complementing rodent repair deficiency, complementation group 1 (ERCC1), result in pre-mature aging, impaired growth, microcephaly and delayed development of the cerebellum...
  31. Spoor M, Nagtegaal A, Ridwan Y, Borgesius N, van Alphen B, van der Pluijm I, et al. Accelerated loss of hearing and vision in the DNA-repair deficient Ercc1(?/-) mouse. Mech Ageing Dev. 2012;133:59-67 pubmed publisher
    ..whether DNA damage can contribute to age-related hearing and vision loss, we investigated hearing and vision in Ercc1(?/-) mutant mice, which are deficient in DNA repair of helix-distorting DNA lesions and interstrand DNA crosslinks...
  32. Theil A, Nonnekens J, Steurer B, Mari P, de Wit J, Lemaître C, et al. Disruption of TTDA results in complete nucleotide excision repair deficiency and embryonic lethality. PLoS Genet. 2013;9:e1003431 pubmed publisher
    ..These findings reveal an essential role of TTDA for life, nucleotide excision repair, and oxidative DNA damage repair and identify Ttda(-/-) cells as a unique class of TFIIH mutants. ..
  33. Liu Y, Chang P, Chao C. CITED2 silencing sensitizes cancer cells to cisplatin by inhibiting p53 trans-activation and chromatin relaxation on the ERCC1 DNA repair gene. Nucleic Acids Res. 2015;43:10760-81 pubmed publisher, we show that silencing of CITED2 using small-hairpin RNA (shCITED2) induced DNA damage and reduction of ERCC1 gene expression in HEK293, HeLa and H1299 cells, even in the absence of cisplatin...
  34. Takayama K, Kawakami Y, Lee S, Greco N, Lavasani M, Mifune Y, et al. Involvement of ERCC1 in the pathogenesis of osteoarthritis through the modulation of apoptosis and cellular senescence. J Orthop Res. 2014;32:1326-32 pubmed publisher
    ..Excision repair cross complementation group 1 (ERCC1) is an endonuclease required for DNA damage repair...
  35. Pérez Oliva A, Lachaud C, Szyniarowski P, Muñoz I, MacArtney T, Hickson I, et al. USP45 deubiquitylase controls ERCC1-XPF endonuclease-mediated DNA damage responses. EMBO J. 2015;34:326-43 pubmed publisher
    ..Here, we identify the deubiquitylase USP45 as a critical DNA repair regulator. USP45 associates with ERCC1, a subunit of the DNA repair endonuclease XPF-ERCC1, via a short acidic motif outside of the USP45 catalytic ..
  36. Mitra D, Fernandez P, Bian L, Song N, Li F, Han G, et al. Smad4 loss in mouse keratinocytes leads to increased susceptibility to UV carcinogenesis with reduced Ercc1-mediated DNA repair. J Invest Dermatol. 2013;133:2609-2616 pubmed publisher
    ..Among genes involved in repairing UV-induced DNA damage, Excision repair cross-complementation group 1 (Ercc1) messenger RNA was significantly reduced in UV-treated K14.Smad4(-/-) skin compared with WT skin...
  37. van Duin M, van Den Tol J, Warmerdam P, Odijk H, Meijer D, Westerveld A, et al. Evolution and mutagenesis of the mammalian excision repair gene ERCC-1. Nucleic Acids Res. 1988;16:5305-22 pubmed
    ..Since the ERCC-1 promoter is devoid of all classical promoter elements this region may be responsible for the low constitutive level of expression in all mouse tissues and stages of embryogenesis examined. ..
  38. Geschwind D, Ou J, Easterday M, Dougherty J, Jackson R, Chen Z, et al. A genetic analysis of neural progenitor differentiation. Neuron. 2001;29:325-39 pubmed
    ..This combination of methods demonstrates the power of using custom microarrays derived from RDA-subtracted libraries for both gene discovery and gene expression analysis in the central nervous system. ..
  39. Verhagen Oldenampsen J, Haanstra J, van Strien P, Valkhof M, Touw I, von Lindern M. Loss of ercc1 results in a time- and dose-dependent reduction of proliferating early hematopoietic progenitors. Anemia. 2012;2012:783068 pubmed publisher
    The endonuclease complex Ercc1/Xpf is involved in interstrand crosslink repair and functions downstream of the Fanconi pathway. Loss of Ercc1 causes hematopoietic defects similar to those seen in Fanconi Anemia...
  40. Andriani G, Faggioli F, Baker D, Dollé M, Sellers R, Hébert J, et al. Whole chromosome aneuploidy in the brain of Bub1bH/H and Ercc1-/Δ7 mice. Hum Mol Genet. 2016;25:755-65 pubmed publisher
    ..and cerebellum of adult and developing brain of Bub1b(H/H) mice, which have a faulty mitotic checkpoint, and Ercc1(-/Δ7) mice, defective in nucleotide excision repair and inter-strand cross-link repair...
  41. Chao C, Strgar F, Tsang M, Peterson P. Effects of swimming exercise on the pathogenesis of acute murine Toxoplasma gondii Me49 infection. Clin Immunol Immunopathol. 1992;62:220-6 pubmed
    ..These data show that swimming exercise is not deleterious to mice acutely infected with T. gondii Me49 and that the more rapid recovery in exercised mice is associated with reduced serum TNF levels. ..
  42. Muñoz P, Blanco R, de Carcer G, Schoeftner S, Benetti R, Flores J, et al. TRF1 controls telomere length and mitotic fidelity in epithelial homeostasis. Mol Cell Biol. 2009;29:1608-25 pubmed publisher
    ..Together, these results demonstrate that TRF1, like TRF2, negatively regulates telomere length in vivo by controlling the action of the XPF nuclease at telomeres; in addition, TRF1 has a unique role in the mitotic spindle checkpoint. ..
  43. Zhu Y, Tchkonia T, Pirtskhalava T, Gower A, Ding H, Giorgadze N, et al. The Achilles' heel of senescent cells: from transcriptome to senolytic drugs. Aging Cell. 2015;14:644-58 pubmed publisher
    ..In vivo, this combination reduced senescent cell burden in chronologically aged, radiation-exposed, and progeroid Ercc1(-/Δ) mice...
  44. Winter A, Dorgan C, Melton D. Expression of a splicing variant in the 5'-UTR of the human ERCC1 gene is not cancer related. Oncogene. 2005;24:2110-3 pubmed cisplatin is increased nucleotide excision repair activity, in particular increased levels of the endonuclease ERCC1. Since 30-40% of ovarian cancers develop resistance to cisplatin after treatment and these tumours are usually ..
  45. Vermeij W, Dollé M, Reiling E, Jaarsma D, Payan Gomez C, Bombardieri C, et al. Restricted diet delays accelerated ageing and genomic stress in DNA-repair-deficient mice. Nature. 2016;537:427-431 pubmed publisher
    Mice deficient in the DNA excision-repair gene Ercc1 (Ercc1?/-) show numerous accelerated ageing features that limit their lifespan to 4-6 months...
  46. Schrader C, Vardo J, Linehan E, Twarog M, Niedernhofer L, Hoeijmakers J, et al. Deletion of the nucleotide excision repair gene Ercc1 reduces immunoglobulin class switching and alters mutations near switch recombination junctions. J Exp Med. 2004;200:321-30 pubmed
    The structure-specific endonuclease ERCC1-XPF is an essential component of the nucleotide excision DNA repair pathway. ERCC1-XPF nicks double-stranded DNA immediately adjacent to 3' single-strand regions...
  47. Paul C, Povey J, Lawrence N, Selfridge J, Melton D, Saunders P. Deletion of genes implicated in protecting the integrity of male germ cells has differential effects on the incidence of DNA breaks and germ cell loss. PLoS ONE. 2007;2:e989 pubmed
    ..We have investigated mice with deletions of ERCC1 (excision repair cross-complementing gene 1), MSH2 (MutS homolog 2, involved in mismatch repair pathway), and p53 (..
  48. Wang J, Cao H, You C, Yuan B, Bahde R, Gupta S, et al. Endogenous formation and repair of oxidatively induced G[8-5 m]T intrastrand cross-link lesion. Nucleic Acids Res. 2012;40:7368-74 pubmed
    ..Additionally, XPA-deficient human brain and mouse liver as well as various types of tissues of ERCC1-deficient mice contained higher levels of d(G[8-5 m]T) but not ROS-induced single-nucleobase lesions than the ..
  49. Vannier J, Pavicic Kaltenbrunner V, Petalcorin M, Ding H, Boulton S. RTEL1 dismantles T loops and counteracts telomeric G4-DNA to maintain telomere integrity. Cell. 2012;149:795-806 pubmed publisher
    ..We propose that RTEL1 performs two distinct functions at telomeres: it disassembles T loops and also counteracts telomeric G4-DNA structures, which together ensure the dynamics and stability of the telomere. ..
  50. Chen J, Xavier S, Moskowitz Kassai E, Chen R, Lu C, Sanduski K, et al. Cathepsin cleavage of sirtuin 1 in endothelial progenitor cells mediates stress-induced premature senescence. Am J Pathol. 2012;180:973-83 pubmed publisher
    ..The proposed mechanism of SIRT1 depletion in stress has all of the attributes of being a paradigm of SIPS of EPCs. ..
  51. Cleaver J. It was a very good year for DNA repair. Cell. 1994;76:1-4 pubmed
  52. Hodskinson M, Silhan J, Crossan G, Garaycoechea J, Mukherjee S, Johnson C, et al. Mouse SLX4 is a tumor suppressor that stimulates the activity of the nuclease XPF-ERCC1 in DNA crosslink repair. Mol Cell. 2014;54:472-84 pubmed publisher
    SLX4 binds to three nucleases (XPF-ERCC1, MUS81-EME1, and SLX1), and its deficiency leads to genomic instability, sensitivity to DNA crosslinking agents, and Fanconi anemia...
  53. Raj D, Jaarsma D, Holtman I, Olah M, Ferreira F, Schaafsma W, et al. Priming of microglia in a DNA-repair deficient model of accelerated aging. Neurobiol Aging. 2014;35:2147-60 pubmed publisher
    ..We have studied this in Ercc1 mutant mice, a DNA repair-deficient mouse model that displays features of accelerated aging in multiple tissues ..
  54. Roh D, Du Y, Gabriele M, Robinson A, Niedernhofer L, Funderburgh J. Age-related dystrophic changes in corneal endothelium from DNA repair-deficient mice. Aging Cell. 2013;12:1122-31 pubmed publisher
    ..whether endogenous DNA damage contributes to the age-related spontaneous loss of CE, we characterized CE in Ercc1(-/?) mice, which have impaired capacity to repair DNA damage and age prematurely. Eyes from 4...
  55. Thompson L, Mooney C, Burkhart Schultz K, Carrano A, Siciliano M. Correction of a nucleotide-excision-repair mutation by human chromosome 19 in hamster-human hybrid cells. Somat Cell Mol Genet. 1985;11:87-92 pubmed
    ..This study demonstrates the mapping of a human repair gene by direct selection of complementing hybrids in the presence of a DNA-damaging agent (mitomycin C). ..
  56. Kamileri I, Karakasilioti I, Sideri A, Kosteas T, Tatarakis A, Talianidis I, et al. Defective transcription initiation causes postnatal growth failure in a mouse model of nucleotide excision repair (NER) progeria. Proc Natl Acad Sci U S A. 2012;109:2995-3000 pubmed publisher
    ..Here, we show that the ERCC1-XPF NER endonuclease assembles on active promoters in vivo and facilitates chromatin modifications for ..
  57. Borgesius N, de Waard M, van der Pluijm I, Omrani A, Zondag G, van der Horst G, et al. Accelerated age-related cognitive decline and neurodegeneration, caused by deficient DNA repair. J Neurosci. 2011;31:12543-53 pubmed publisher
    ..Here we take advantage of Ercc1(?/-) mutant mice, which are impaired in DNA nucleotide excision repair, interstrand crosslink repair, and double-..
  58. Nevedomskaya E, Meissner A, Goraler S, de Waard M, Ridwan Y, Zondag G, et al. Metabolic profiling of accelerated aging ERCC1 d/- mice. J Proteome Res. 2010;9:3680-7 pubmed publisher
    ..As there are many causative links between DNA repair deficiency and aging, we studied the ERCC1(d/-) mouse, which has a modified ERCC1 gene, involved in the Nucleotide Excision Repair, and as a result has a ..
  59. Durik M, Kavousi M, van der Pluijm I, Isaacs A, Cheng C, Verdonk K, et al. Nucleotide excision DNA repair is associated with age-related vascular dysfunction. Circulation. 2012;126:468-78 pubmed publisher
    ..In mice with genomic instability resulting from the defective nucleotide excision repair genes ERCC1 and XPD (Ercc1(d/-) and Xpd(TTD) mice), we explored age-dependent vascular function compared with that in wild-..