{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T05:38:05Z","timestamp":1777873085155,"version":"3.51.4"},"reference-count":161,"publisher":"Frontiers Media SA","issue":"6","license":[{"start":{"date-parts":[[2018,3,1]],"date-time":"2018-03-01T00:00:00Z","timestamp":1519862400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Exp Biol Med (Maywood)"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:p>Nonerythroid \u03b1II-spectrin is a structural protein whose roles in the nucleus have just begun to be explored. \u03b1II-spectrin is an important component of the nucleoskelelton and has both structural and non-structural functions. Its best known role is in repair of DNA ICLs both in genomic and telomeric DNA. \u03b1II-spectrin aids in the recruitment of repair proteins to sites of damage and a proposed mechanism of action is presented. It interacts with a number of different groups of proteins in the nucleus, indicating it has roles in additional cellular functions. \u03b1II-spectrin, in its structural role, associates\/co-purifies with proteins important in maintaining the architecture and mechanical properties of the nucleus such as lamin, emerin, actin, protein 4.1, nuclear myosin, and SUN proteins. It is important for the resilience and elasticity of the nucleus. Thus, \u03b1II-spectrin\u2019s role in cellular functions is complex due to its structural as well as non-structural roles and understanding the consequences of a loss or deficiency of \u03b1II-spectrin in the nucleus is a significant challenge. In the bone marrow failure disorder, Fanconi anemia, there is a deficiency in \u03b1II-spectrin and, among other characteristics, there is defective DNA repair, chromosome instability, and congenital abnormalities. One may speculate that a deficiency in \u03b1II-spectrin plays an important role not only in the DNA repair defect but also in the congenital anomalies observed in Fanconi anemia , particularly since \u03b1II-spectrin has been shown to be important in embryonic development in a mouse model. The dual roles of \u03b1II-spectrin in the nucleus in both structural and non-structural functions make this an extremely important protein which needs to be investigated further. Such investigations should help unravel the complexities of \u03b1II-spectrin\u2019s interactions with other nuclear proteins and enhance our understanding of the pathogenesis of disorders, such as Fanconi anemia , in which there is a deficiency in \u03b1II-spectrin.<\/jats:p>\n                  <jats:sec>\n                    <jats:title>Impact statement<\/jats:title>\n                    <jats:p>The nucleoskeleton is critical for maintaining the architecture and functional integrity of the nucleus. Nonerythroid \u03b1-spectrin (\u03b1IISp) is an essential nucleoskeletal protein; however, its interactions with other structural and non-structural nuclear proteins and its functional importance in the nucleus have only begun to be explored. This review addresses these issues. It describes \u03b1IISp\u2019s association with DNA repair proteins and at least one proposed mechanism of action for its role in DNA repair. Specific interactions of \u03b1IISp with other nucleoskeletal proteins as well as its important role in the biomechanical properties of the nucleus are reviewed. The consequences of loss of \u03b1IISp, in disorders such as Fanconi anemia, are examined, providing insights into the profound impact of this loss on critical processes known to be abnormal in FA, such as development, carcinogenesis, cancer progression and cellular functions dependent upon \u03b1IISp\u2019s interactions with other nucleoskeletal proteins.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/1535370218763563","type":"journal-article","created":{"date-parts":[[2018,3,20]],"date-time":"2018-03-20T07:33:34Z","timestamp":1521531214000},"page":"507-524","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":19,"title":["Spectrin and its interacting partners in nuclear structure and function"],"prefix":"10.3389","volume":"243","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4503-0890","authenticated-orcid":false,"given":"Muriel W","family":"Lambert","sequence":"first","affiliation":[{"name":"Department 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