The fanconi anaemia/BRCA pathway

Alan D. D'Andrea; Markus Grompe

doi:10.1038/nrc970

The fanconi anaemia/BRCA pathway

Alan D. D'Andrea, Markus Grompe

Pediatrics

Research output: Contribution to journal › Review article › peer-review

685 Scopus citations

Abstract

Fanconi anaemia (FA) is a rare genetic cancer-susceptibility syndrome that is characterized by congenital abnormalities, bone-marrow failure and cellular sensitivity to DNA crosslinking agents. Seven FA-associated genes have recently been cloned, and their products were found to interact with well-known DNA-damage-response proteins, including BRCA1, ATM and NBS1. The FA proteins could therefore be involved in the cell-cycle checkpoint and DNA-repair pathways. Recent studies implicate the FA proteins in the process of repairing chromosome defects that occur during homologous recombination, and disruption of the FA genes results in chromosome instability - a common feature of many human cancers.

Original language	English (US)
Pages (from-to)	23-34
Number of pages	12
Journal	Nature Reviews Cancer
Volume	3
Issue number	1
DOIs	https://doi.org/10.1038/nrc970
State	Published - Jan 2003

ASJC Scopus subject areas

Oncology
Cancer Research

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title = "The fanconi anaemia/BRCA pathway",

abstract = "Fanconi anaemia (FA) is a rare genetic cancer-susceptibility syndrome that is characterized by congenital abnormalities, bone-marrow failure and cellular sensitivity to DNA crosslinking agents. Seven FA-associated genes have recently been cloned, and their products were found to interact with well-known DNA-damage-response proteins, including BRCA1, ATM and NBS1. The FA proteins could therefore be involved in the cell-cycle checkpoint and DNA-repair pathways. Recent studies implicate the FA proteins in the process of repairing chromosome defects that occur during homologous recombination, and disruption of the FA genes results in chromosome instability - a common feature of many human cancers.",

author = "D'Andrea, {Alan D.} and Markus Grompe",

note = "Funding Information: We apologize to those authors whose work is not cited owing to space constraints. A.D.D. is a Doris Duke Distinguished Clinical Scientist and is supported by National Institutes of Health grants. M.G. is supported by a National Institutes of Health grant.",

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N1 - Funding Information: We apologize to those authors whose work is not cited owing to space constraints. A.D.D. is a Doris Duke Distinguished Clinical Scientist and is supported by National Institutes of Health grants. M.G. is supported by a National Institutes of Health grant.

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N2 - Fanconi anaemia (FA) is a rare genetic cancer-susceptibility syndrome that is characterized by congenital abnormalities, bone-marrow failure and cellular sensitivity to DNA crosslinking agents. Seven FA-associated genes have recently been cloned, and their products were found to interact with well-known DNA-damage-response proteins, including BRCA1, ATM and NBS1. The FA proteins could therefore be involved in the cell-cycle checkpoint and DNA-repair pathways. Recent studies implicate the FA proteins in the process of repairing chromosome defects that occur during homologous recombination, and disruption of the FA genes results in chromosome instability - a common feature of many human cancers.

AB - Fanconi anaemia (FA) is a rare genetic cancer-susceptibility syndrome that is characterized by congenital abnormalities, bone-marrow failure and cellular sensitivity to DNA crosslinking agents. Seven FA-associated genes have recently been cloned, and their products were found to interact with well-known DNA-damage-response proteins, including BRCA1, ATM and NBS1. The FA proteins could therefore be involved in the cell-cycle checkpoint and DNA-repair pathways. Recent studies implicate the FA proteins in the process of repairing chromosome defects that occur during homologous recombination, and disruption of the FA genes results in chromosome instability - a common feature of many human cancers.

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The fanconi anaemia/BRCA pathway

Abstract

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