Spectrin: Its Role in DNA Repair

FANCA
DOI: 10.1096/fasebj.20.5.a868-d Publication Date: 2021-06-22T00:06:55Z
ABSTRACT
Studies in our laboratory demonstrate a role for nonerythroid α spectrin (αSpII∑*) human cell nuclei as well new function it the cell, an involvement DNA repair, particular, repair of interstrand cross-links. In cells treated with cross-linking agent, αSpII∑* co-localizes damage-induced nuclear foci XPF, protein involved cross-link repair. It also co-immunoprecipitates XPF. HeLa and purified bovine brain bind to containing Also, anti-αSpII∑* decreases levels incisions produced by normal proteins on cross-linked DNA. from patients Fanconi anemia (FA), genetic disorder characterized bone marrow failure, predisposition cancer defect ability cross-links, we have shown that there is deficiency αSpII∑*. This correlates these cells. FA, complementation group A cells, loss formation XPF after damage which siRNA-mediated silencing αII gene expression results reduction decreased survival treatment agent. Based studies, proposed model needed where acts scaffold recruitment alignment at sites damage. FA αSpII∑*, this are defective. Supported NIH Grants R01 HL054860 ES011298.
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