Rola białek szlaku niedokrwistości Fanconiego w naprawie DNA i utrzymaniu stabilności genomu

Aleksandra M. Koczorowska 1, Aneta Białkowska 1, Katarzyna Kluzek 1, Małgorzata Z. Zdzienicka 1
1 - Katedra i Zakład Genetyki Molekularnej Komórki, Uniwersytet Mikołaja Kopernika w Toruniu, Collegium Medicum im. L. Rydygiera w Bydgoszczy
Postepy Hig Med Dosw
2014; 68 459-472
ICID: 1101567
Article type: Review article
 
 
The Fanconi anemia (FA) pathway is one of the DNA repair systems involved in removal of DNA crosslinks. Proteins which belong to this pathway are crucial to the protection of genetic information, whereas disturbances in their function have serious implications for the whole organism. Biallelic mutations in FA genes are the cause of Fanconi anemia – a genetic disease which manifests itself through numerous congenital abnormalities, chromosomal instability and increased predisposition to cancer. The FA pathway is composed of fifteen proteins. Eight of them, in the presence of DNA interstrand crosslinks (ICLs), form a nuclear core complex responsible for monoubiquitination of FANCD2 and FANCI, which is a key step of ICL repair. FA proteins which are not involved in the monoubiquitination step participate in repair of DNA double strand breaks via homologous recombination. Some of the FA proteins, besides having a direct role in the repair of DNA damage, are engaged in replication, cell cycle control and mitosis. The unperturbed course of those processes determines the maintenance of genome stability.
DOI: 10.5604/17322693.1101567
PMID 24864098 - kliknij tu by zobaczyć artykuł w bazie danych PubMed
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