Fanconi anemia (cross)linked to DNA repair

Laura J Niedernhofer; Astrid S Lalai; Jan H J Hoeijmakers

doi:10.1016/j.cell.2005.12.009

Fanconi anemia (cross)linked to DNA repair

Cell. 2005 Dec 29;123(7):1191-8. doi: 10.1016/j.cell.2005.12.009.

Authors

Laura J Niedernhofer¹, Astrid S Lalai, Jan H J Hoeijmakers

Affiliation

¹ Center for Biomedical Genetics, Medical Genetic Center, Department of Cell Biology and Genetics, Erasmus Medical Center, P.O. Box 1738, 3000 DR Rotterdam, The Netherlands.

PMID: 16377561
DOI: 10.1016/j.cell.2005.12.009

Abstract

Fanconi anemia is characterized by hypersensitivity to DNA interstrand crosslinks (ICLs) and susceptibility to tumor formation. Despite the identification of numerous Fanconi anemia (FANC) genes, the mechanism by which proteins encoded by these genes protect a cell from DNA interstrand crosslinks remains unclear. The recent discovery of two DNA helicases that, when defective, cause Fanconi anemia tips the balance in favor of the direct involvement of the FANC proteins in DNA repair and the bypass of DNA lesions.

Publication types

Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review

MeSH terms

Basic-Leucine Zipper Transcription Factors / genetics
DNA Helicases / genetics*
DNA Helicases / physiology*
DNA Repair / genetics*
Fanconi Anemia / etiology*
Fanconi Anemia / genetics*
Fanconi Anemia Complementation Group Proteins / genetics*
Fanconi Anemia Complementation Group Proteins / physiology
Humans

Substances

BACH1 protein, human
Basic-Leucine Zipper Transcription Factors
Fanconi Anemia Complementation Group Proteins
DNA Helicases

Abstract

Publication types

MeSH terms

Substances

Grants and funding