論文 - 詳細
| RRC ID | 6440 |
|---|---|
| 著者 | Youds JL, Barber LJ, Boulton SJ. |
| タイトル | C. elegans: a model of Fanconi anemia and ICL repair. |
| ジャーナル | Mutat Res |
| Abstract |
Fanconi anemia (FA) is a severe recessive disorder with a wide range of clinical manifestations [M. Levitus, H. Joenje, J.P. de Winter, The Fanconi anemia pathway of genomic maintenance, Cell Oncol. 28 (2006) 3-29]. In humans, 13 complementation groups have been identified to underlie FA: A, B, C, D1, D2, E, F, G, I, J, L, M, and N [W. Wang, Emergence of a DNA-damage response network consisting of Fanconi anaemia and BRCA proteins, Nat. Rev. Genet. 8 (2007) 735-748]. Cells defective for any of these genes display chromosomal aberrations and sensitivity to DNA interstrand cross-links (ICLs). It has therefore been suggested that the 13 FA proteins constitute a pathway for the repair of ICLs, and that a deficiency in this repair process causes genomic instability leading to the different clinical phenotypes. However, the exact nature of this repair pathway, or even whether all 13 FA proteins are involved at some stage of a linear repair process, remains to be defined. Undoubtedly, the recent identification and characterisation of FA homologues in model organisms, such as Caenorhabditis elegans, will help facilitate an understanding of the function of the FA proteins by providing new analytical tools. To date, sequence homologues of five FA genes have been identified in C. elegans. Three of these homologues have been confirmed: brc-2 (FANCD1/BRCA2), fcd-2 (FANCD2), and dog-1 (FANCJ/BRIP1); and two remain to be characterised: W02D3.10 (FANCI) and drh-3 (FANCM). Here we review how the nematode can be used to study FA-associated DNA repair, focusing on what is known about the ICL repair genes in C. elegans and which important questions remain for the field. |
| 巻・号 | 668(1-2) |
| ページ | 103-16 |
| 公開日 | 2009-7-31 |
| DOI | 10.1016/j.mrfmmm.2008.11.007 |
| PII | S0027-5107(08)00301-1 |
| PMID | 19059419 |
| MeSH | Animals Caenorhabditis elegans / genetics* Cross-Linking Reagents / pharmacology* DNA Repair* Fanconi Anemia / genetics* Fanconi Anemia Complementation Group Proteins / metabolism Genomic Instability Models, Animal* |
| IF | 2.463 |
| 引用数 | 20 |
| WOS 分野 | TOXICOLOGY BIOTECHNOLOGY & APPLIED MICROBIOLOGY GENETICS & HEREDITY |
| オルトメトリクス指標 |
オルトメトリクス指標項目
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| 各媒体での言及数の合計 | 0 |
| 過去6か月間でのオルトメトリクス指標の変動値 | 0.0 |
| リソース情報 | |
| 線虫 | tm3081 tm1096 tm1298 |