The Fanconi anemia pathway and ubiquitin

被引：52

作者：

Jacquemont, Celine

Taniguchi, Toshiyasu ^{[1
]}

机构：

[1] Fred Hutchinson Canc Res Ctr, Div Human Biol, Seattle, WA 98109 USA

来源：

BMC BIOCHEMISTRY | 2007年 / 8卷

关键词：

D O I：

10.1186/1471-2091-8-S1-S10

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Fanconi anemia (FA) is a rare genetic disorder characterized by aplastic anemia, cancer/leukemia susceptibility and cellular hypersensitivity to DNA crosslinking agents, such as cisplatin. To date, 12 FA gene products have been identified, which cooperate in a common DNA damage-activated signaling pathway regulating DNA repair (the FA pathway). Eight FA proteins form a nuclear complex harboring E3 ubiquitin ligase activity (the FA core complex) that, in response to DNA damage, mediates the monoubiquitylation of the FA protein FANCD2. Monoubiquitylated FANCD2 colocalizes in nuclear foci with proteins involved in DNA repair, including BRCA1, FANCD1/BRCA2, FANCN/PALB2 and RAD51. All these factors are required for cellular resistance to DNA crosslinking agents. The inactivation of the FA pathway has also been observed in a wide variety of human cancers and is implicated in the sensitivity of cancer cells to DNA crosslinking agents. Drugs that inhibit the FA pathway may be useful chemosensitizers in the treatment of cancer.

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页数：10

相关论文

共 137 条

[11] Regulation of the Fanconi anemia group C protein through proteolytic modification [J].

Brodeur, I ;

Goulet, I ;

Tremblay, CS ;

Charbonneau, C ;

Delisle, MC ;

Godin, C ;

Huard, C ;

Khandjian, EW ;

Buchwald, M ;

Levesque, G ;

Carreau, M .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2004, 279 (06) :4713-4720

[12] BACH1, a novel helicase-like protein, interacts directly with BRCA1 and contributes to its DNA repair function [J].

Cantor, SB ;

Bell, DW ;

Ganesan, S ;

Kass, EM ;

Drapkin, R ;

Grossman, S ;

Wahrer, DCR ;

Sgroi, DC ;

Lane, WS ;

Haber, DA ;

Livingston, DM .

CELL, 2001, 105 (01) :149-160

[13] Not-so-novel phenotypes in the Fanconi anemia group D2 mouse model [J].

Carreau, M .

BLOOD, 2004, 103 (06) :2430-2430

[14] Short-term granulocyte colony-stimulating factor and erythropoietin treatment enhances hematopoiesis and survival in the mitomycin C-conditioned Fancc-1- mouse model, while long-term treatment is ineffective [J].

Carreau, M ;

Liu, L ;

Gan, OI ;

Hitzler, JK ;

Dick, JE ;

Buchwald, M .

BLOOD, 2002, 100 (04) :1499-1501

[15] Bone marrow failure in the Fanconi anemia group C mouse model after DNA damage [J].

Carreau, M ;

Gan, OI ;

Liu, LL ;

Doedens, M ;

McKerlie, C ;

Dick, JE ;

Buchwald, M .

BLOOD, 1998, 91 (08) :2737-2744

[16] Inactivation of Fac in mice produces inducible chromosomal instability and reduced fertility reminiscent of Fanconi anaemia [J].

Chen, M ;

Tomkins, DJ ;

Auerbach, W ;

McKerlie, C ;

Youssoufian, H ;

Liu, L ;

Gan, O ;

Carreau, M ;

Auerbach, A ;

Groves, T ;

Guidos, CJ ;

Freedman, MH ;

Cross, J ;

Percy, DH ;

Dick, JE ;

Joyner, AL ;

Buchwald, M .

NATURE GENETICS, 1996, 12 (04) :448-451

[17] The FA/BRCA pathway is involved in melphalan-induced DNA interstrand cross-link repair and accounts for melphalan resistance in multiple myeloma cells [J].

Chen, Q ;

Van der Sluis, PC ;

Boulware, D ;

Hazlehurst, LA ;

Dalton, WS .

BLOOD, 2005, 106 (02) :698-705

[18]

Cheng AL, 2001, ANTICANCER RES, V21, P2895

[19] Mice with a targeted disruption of the Fanconi anemia homolog Fanca [J].

Cheng, NC ;

van de Vrugt, HJ ;

van der Valk, MA ;

Oostra, AB ;

Krimpenfort, P ;

de Vries, Y ;

Joenje, H ;

Berns, A ;

Arwert, F .

HUMAN MOLECULAR GENETICS, 2000, 9 (12) :1805-1811

[20] Chemosensitization to cisplatin by inhibitors of the Fanconi anemia/BRCA pathway [J].

Chirnomas, D ;

Taniguchi, T ;

de la Vega, M ;

Vaidya, AP ;

Vasserman, M ;

Hartman, AR ;

Kennedy, R ;

Foster, R ;

Mahoney, J ;

Seiden, MV ;

D'Andrea, AD .

MOLECULAR CANCER THERAPEUTICS, 2006, 5 (04) :952-961

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