Assembling an orchestra

Fanconi anemia pathway of DNA repair

Fenghua Yuan, Limin Song, Liangyue Qian, Jennifer Hu, Yanbin Zhang

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Fanconi anemia (FA) is a recessive genetic disorder characterized by developmental defects, bone marrow failure, and cancer susceptibility. The complete set of FA genes has only been identified recently and seems to be uniquely conserved among vertebrates. Fanconi anemia proteins have been implicated in the repair of interstrand DNA crosslinks that block DNA replication and transcription. Although all thirteen FA complementation groups show similar clinical and cellular phenotypes, approximately 85% of patients presented defective FANCA, FANCC, or FANCG. The established DNA interacting components (FANCM, FANCI, FANCD2, and FANCJ) account only for ~5% of all FA patients, an observation that raises doubt concerning the roles of FA proteins in DNA repair. In recent years, rapid progress in the area of FA research has provided great insights into the critical roles of FA proteins in DNA repair. However, many FA proteins do not have identifiable domains to indicate how they contribute to biological processes, particularly DNA repair. Therefore, future biochemical studies are warranted to understand the biological functions of FA proteins and their implications in human diseases.

Original languageEnglish
Pages (from-to)1131-1149
Number of pages19
JournalFrontiers in Bioscience
Volume15
Issue number3
DOIs
StatePublished - Jun 1 2010

Fingerprint

Fanconi Anemia Complementation Group Proteins
Fanconi Anemia
DNA Repair
Repair
DNA
Biological Phenomena
Bone Neoplasms
Inborn Genetic Diseases
DNA Replication
Transcription
Vertebrates
Bone Marrow
Observation
Bone
Phenotype
Genes
Defects
Research

Keywords

  • Cancer
  • Dna repair
  • Fanconi anemia
  • Homologous recombination
  • Icl incision
  • Interstrand crosslink
  • Replication
  • Review
  • Translesion synthesis

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Microbiology(all)
  • Medicine(all)

Cite this

Assembling an orchestra : Fanconi anemia pathway of DNA repair. / Yuan, Fenghua; Song, Limin; Qian, Liangyue; Hu, Jennifer; Zhang, Yanbin.

In: Frontiers in Bioscience, Vol. 15, No. 3, 01.06.2010, p. 1131-1149.

Research output: Contribution to journalArticle

Yuan, Fenghua ; Song, Limin ; Qian, Liangyue ; Hu, Jennifer ; Zhang, Yanbin. / Assembling an orchestra : Fanconi anemia pathway of DNA repair. In: Frontiers in Bioscience. 2010 ; Vol. 15, No. 3. pp. 1131-1149.
@article{3d03c7e2565f488f9882048d11978358,
title = "Assembling an orchestra: Fanconi anemia pathway of DNA repair",
abstract = "Fanconi anemia (FA) is a recessive genetic disorder characterized by developmental defects, bone marrow failure, and cancer susceptibility. The complete set of FA genes has only been identified recently and seems to be uniquely conserved among vertebrates. Fanconi anemia proteins have been implicated in the repair of interstrand DNA crosslinks that block DNA replication and transcription. Although all thirteen FA complementation groups show similar clinical and cellular phenotypes, approximately 85{\%} of patients presented defective FANCA, FANCC, or FANCG. The established DNA interacting components (FANCM, FANCI, FANCD2, and FANCJ) account only for ~5{\%} of all FA patients, an observation that raises doubt concerning the roles of FA proteins in DNA repair. In recent years, rapid progress in the area of FA research has provided great insights into the critical roles of FA proteins in DNA repair. However, many FA proteins do not have identifiable domains to indicate how they contribute to biological processes, particularly DNA repair. Therefore, future biochemical studies are warranted to understand the biological functions of FA proteins and their implications in human diseases.",
keywords = "Cancer, Dna repair, Fanconi anemia, Homologous recombination, Icl incision, Interstrand crosslink, Replication, Review, Translesion synthesis",
author = "Fenghua Yuan and Limin Song and Liangyue Qian and Jennifer Hu and Yanbin Zhang",
year = "2010",
month = "6",
day = "1",
doi = "10.2741/3665",
language = "English",
volume = "15",
pages = "1131--1149",
journal = "Frontiers in Bioscience - Landmark",
issn = "1093-9946",
publisher = "Frontiers in Bioscience",
number = "3",

}

TY - JOUR

T1 - Assembling an orchestra

T2 - Fanconi anemia pathway of DNA repair

AU - Yuan, Fenghua

AU - Song, Limin

AU - Qian, Liangyue

AU - Hu, Jennifer

AU - Zhang, Yanbin

PY - 2010/6/1

Y1 - 2010/6/1

N2 - Fanconi anemia (FA) is a recessive genetic disorder characterized by developmental defects, bone marrow failure, and cancer susceptibility. The complete set of FA genes has only been identified recently and seems to be uniquely conserved among vertebrates. Fanconi anemia proteins have been implicated in the repair of interstrand DNA crosslinks that block DNA replication and transcription. Although all thirteen FA complementation groups show similar clinical and cellular phenotypes, approximately 85% of patients presented defective FANCA, FANCC, or FANCG. The established DNA interacting components (FANCM, FANCI, FANCD2, and FANCJ) account only for ~5% of all FA patients, an observation that raises doubt concerning the roles of FA proteins in DNA repair. In recent years, rapid progress in the area of FA research has provided great insights into the critical roles of FA proteins in DNA repair. However, many FA proteins do not have identifiable domains to indicate how they contribute to biological processes, particularly DNA repair. Therefore, future biochemical studies are warranted to understand the biological functions of FA proteins and their implications in human diseases.

AB - Fanconi anemia (FA) is a recessive genetic disorder characterized by developmental defects, bone marrow failure, and cancer susceptibility. The complete set of FA genes has only been identified recently and seems to be uniquely conserved among vertebrates. Fanconi anemia proteins have been implicated in the repair of interstrand DNA crosslinks that block DNA replication and transcription. Although all thirteen FA complementation groups show similar clinical and cellular phenotypes, approximately 85% of patients presented defective FANCA, FANCC, or FANCG. The established DNA interacting components (FANCM, FANCI, FANCD2, and FANCJ) account only for ~5% of all FA patients, an observation that raises doubt concerning the roles of FA proteins in DNA repair. In recent years, rapid progress in the area of FA research has provided great insights into the critical roles of FA proteins in DNA repair. However, many FA proteins do not have identifiable domains to indicate how they contribute to biological processes, particularly DNA repair. Therefore, future biochemical studies are warranted to understand the biological functions of FA proteins and their implications in human diseases.

KW - Cancer

KW - Dna repair

KW - Fanconi anemia

KW - Homologous recombination

KW - Icl incision

KW - Interstrand crosslink

KW - Replication

KW - Review

KW - Translesion synthesis

UR - http://www.scopus.com/inward/record.url?scp=77956621425&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77956621425&partnerID=8YFLogxK

U2 - 10.2741/3665

DO - 10.2741/3665

M3 - Article

VL - 15

SP - 1131

EP - 1149

JO - Frontiers in Bioscience - Landmark

JF - Frontiers in Bioscience - Landmark

SN - 1093-9946

IS - 3

ER -