The Fanconi anemia pathway and DNA interstrand cross-link repair.

Abstract:

:Fanconi anemia (FA) is an autosomal or X-linked recessive disorder characterized by chromosomal instability, bone marrow failure, cancer susceptibility, and a profound sensitivity to agents that produce DNA interstrand cross-link (ICL). To date, 15 genes have been identified that, when mutated, result in FA or an FA-like syndrome. It is believed that cellular resistance to DNA interstrand cross-linking agents requires all 15 FA or FA-like proteins. Here, we review our current understanding of how these FA proteins participate in ICL repair and discuss the molecular mechanisms that regulate the FA pathway to maintain genome stability.

journal_name

Protein Cell

journal_title

Protein & cell

authors

Su X,Huang J

doi

10.1007/s13238-011-1098-y

subject

Has Abstract

pub_date

2011-09-01 00:00:00

pages

704-11

issue

9

eissn

1674-800X

issn

1674-8018

journal_volume

2

pub_type

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