The Fanconi anemia gene product FANCF is a flexible adaptor protein

被引:52
作者
Léveillé, F
Blom, E
Medhurst, AL
Bier, P
Laghmani, EH
Johnson, M
Rooimans, MA
Sobeck, A
Waisfisz, Q
Arwert, F
Patel, KJ
Hoatlin, ME
Joenje, H
de Winter, JP
机构
[1] Vrije Univ Amsterdam Med Ctr, Dept Clin Genet & Human Genet, NL-1081 BT Amsterdam, Netherlands
[2] Guys Kings & St Thomas Sch Med, Div Genet & Dev, London SE1 9RT, England
[3] MRC, Mol Biol Lab, Cambridge CB2 2QH, England
[4] Oregon Hlth & Sci Univ, Div Mol Med, Portland, OR 97239 USA
关键词
D O I
10.1074/jbc.M407034200
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
The Fanconi anemia (FA) protein FANCF is an essential component of a nuclear core complex that protects the genome against chromosomal instability, but the specific function of FANCF is still poorly understood. Based upon the homology between human and Xenopus laevis FANCF, we carried out an extensive mutagenesis study to examine which domains are functionally important and to gain more insight into the function of FANCF. In contrast to previous suggestions, we show that FANCF does not have a ROM-like function. We found that the C terminus of FANCF interacts directly with FANCG and allows the assembly of other FA proteins into a stable complex. The N terminus appears to stabilize the interaction with FANCA and FANCG and is essential for the binding of the FANCC/FANCE subcomplex. We identified several important amino acids in this N-terminal region but, surprisingly, many amino acid changes failed to affect the function of the FANCF protein. Our data demonstrate that FANCF acts as a flexible adaptor protein that plays a key role in the proper assembly of the FA core complex.
引用
收藏
页码:39421 / 39430
页数:10
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