Towards a Molecular Understanding of the Fanconi Anemia Core Complex
Joint Authors
Walden, Helen
Hodson, Charlotte
Source
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-10, 10 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-05-22
Country of Publication
Egypt
No. of Pages
10
Main Subjects
Abstract EN
Fanconi Anemia (FA) is a genetic disorder characterized by the inability of patient cells to repair DNA damage caused by interstrand crosslinking agents.
There are currently 14 verified FA genes, where mutation of any single gene prevents repair of DNA interstrand crosslinks (ICLs).
The accumulation of ICL damage results in genome instability and patients having a high predisposition to cancers.
The key event of the FA pathway is dependent on an eight-protein core complex (CC), required for the monoubiquitination of each member of the FANCD2-FANCI complex.
Interestingly, the majority of patient mutations reside in the CC.
The molecular mechanisms underlying the requirement for such a large complex to carry out a monoubiquitination event remain a mystery.
This paper documents the extensive efforts of researchers so far to understand the molecular roles of the CC proteins with regard to its main function in the FA pathway, the monoubiquitination of FANCD2 and FANCI.
American Psychological Association (APA)
Hodson, Charlotte& Walden, Helen. 2012. Towards a Molecular Understanding of the Fanconi Anemia Core Complex. Anemia،Vol. 2012, no. 2012, pp.1-10.
https://search.emarefa.net/detail/BIM-508724
Modern Language Association (MLA)
Hodson, Charlotte& Walden, Helen. Towards a Molecular Understanding of the Fanconi Anemia Core Complex. Anemia No. 2012 (2012), pp.1-10.
https://search.emarefa.net/detail/BIM-508724
American Medical Association (AMA)
Hodson, Charlotte& Walden, Helen. Towards a Molecular Understanding of the Fanconi Anemia Core Complex. Anemia. 2012. Vol. 2012, no. 2012, pp.1-10.
https://search.emarefa.net/detail/BIM-508724
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-508724