Bioscience Chapter Database :: 3635 Chapters Now Online

Chapter category: Hematology

The FANC Genome Surveillance Complex

This chapter appears in the following book:

Molecular Mechanisms of Fanconi Anemia

Edited by: Shamim I. Ahmad and Sandra H. Kirk
ISBN: 0-387-31972-7
» Get more information about this book at landesbioscience.com «

Chapter authors:
Takayuki Yamashita


[+] view image
FA is an autosomal recessive genetic disorder characterized by progressive bone marrow failure, congenital anomalies and susceptibility to hematological and solid malignancies. The most consistent feature of FA cells is their hypersensitivity to DNA cross-linking agents such as diepoxybutane (DEB) and mitomycin C (MMC). These chemicals at low concentrations induce higher levels of chromosome breakage and cell death in FA cell lines than in control cells. Genetic heterogeneity of FA was shown by complementation analyses based on the cellular hypersensitivity to DNA cross-linking agents (see chapter 3). Since initial identification of two different groups,1 by 1992 the number of complementation groups increased to four.2 The first FA gene cloned was of complementation group C found to be involved in the correction of DNA cross-links and termed FACC,3 which later renamed as FANCC.

» Access chapter for $19



Additional chapters from this book:

Fanconi Anaemia and Oxidative Stress: Cellular and Clinical Phenotypes

Giovanni Pagano and Shamim I. Ahmad

The cellular and clinical phenotypes of Fanconi Anaemia (FA) have been associated with a set of redox abnormalities using evidence arising from in vitro, in vivo and molecular studies. The available i...

Clinical Features of Fanconi Anaemia

A. Malcolm R. Taylor

Fanconi anaemia is an autosomal recessive disorder in which patients develop bone mar row failure and aplastic anaemia but this can occur at widely differing ages from the first year to age 12 years...

The Genetic Basis of Fanconi Anemia

Grover C. Bagby, Jr.

Seventy-five years ago, Dr. Guido Fanconi reported three siblings who exhibited both congenital defects and aplastic anemia.1 Since then, we have learned that Fanconi anemia (FA) is a rare multigeni...

The FANC B, E, F and G Genes and their Products

Filippo Rosselli

The rare autosomal recessive syndrome Fanconi anemia (FA) leads to bone marrow failure and malignancy predisposition. Moreover, patients may present with several congenital anomalies of the skeleton...

The FANCA Gene and its Products

L. S. Haneline

Fanconi anemia (FA), type A is the most common FA complementation type with muta tions in the FANCA gene accounting for approximately 65% of cases.1,2 The FANCA protein is recognized as an integral ...

FANCD1/BRCA2 and FANCD2

Gary M.Kupfer

The field of FA was both excited as well as surprised when the first cloned genes encoded proteins that resembled no known protein motif. Several binding proteins were described, yet no biochemical ...

The FANC Genome Surveillance Complex

Takayuki Yamashita

FA is an autosomal recessive genetic disorder characterized by progressive bone marrow failure, congenital anomalies and susceptibility to hematological and solid malignancies. The most consistent f...

Mutational Analyses of Fanconi Anemia Genes in Japanese Patients

Akira Tachibana

Fanconi anemia (FA) is an autosomal recessive disorder characterized by a progressive pancytopenia associated with congenital anomalies and high predisposition to malig nancies.1 Certain FA cell lin...

Therapy for Fanconi Anemia

Madeleine Carreau

Treatment of the hematological manifestation in Fanconi anemia is first supportive (trans fusions) with attempts to stimulate hematopoiesis with either androgens, usually oxymetholone, or the hemato...

Other Proteins and Their Interactions With FA Gene Products

Tetsuya Otsuki, Johnson M. Liu

Fanconi anemia (FA) is a genetically heterogeneous disorder, consisting of at least eight complementation groups (FA-A, -B, -C, -D1, -D2, -E, -F and –G).1-3 To date, seven FA genes, FANC-A, -C, -D1 ...

The FANCC Gene and its Products

Susan M. Gordon and Manuel Buchwald

Fanconi anaemia (FA) is an autosomal recessive disorder characterized by progressive pancytopaenia and predisposition to malignancy, often accompanied by congenital malformations. The cellular pheno...


SIGN IN

Email:


Password:


lost password?




[ Home | Authors | Editors | Custom Books | Chapter Reprints | Subscribe | Contact | Biotoons ]