Human α Spectrin II and the Fanconi Anemia Proteins FANCA and FANCC Interact to Form a Nuclear Complex

• DOI: 10.1074/jbc.274.46.32904
• Publication Date: 2002-07-26T14:59:58Z
• Authors: Laura W. McMahon; Christopher E. Walsh; Muriel W. Lambert
• Keywords: FANCA; FANCD2; Bone marrow failure

Fanconi anemia (FA) is a genetic disorder characterized by bone marrow failure, congenital abnormalities, cancer susceptibility, and marked cellular hypersensitivity to DNA interstrand cross-linking agents, which correlates with defect in ability repair this type of damage. We have previously identified an approximately 230-kDa protein present nuclear complex normal human lymphoblastoid cells that involved cross-links shows reduced levels FA-A cell nuclei. The FANCA gene appears play role the stability or expression protein. now show p230 well known structural protein, α spectrin II (αSpIIΣ*), αSpIIΣ* are not only significantly but also FA-B, FA-C FA-D (<i>i.e.</i> all FA lines tested), suggesting for these proteins αSpIIΣ*. These studies forms nucleus FANCC proteins. may thus act as scaffold align enhance interactions between repair. results suggest represents there deficiency