Nijmegen Breakage Syndrome fibroblasts and iPSCs: cellular models for uncovering disease-associated signaling pathways and establishing a screening platform for anti-oxidants
Abstract Nijmegen Breakage Syndrome (NBS) is associated with cancer predisposition, premature aging, immune deficiency, microcephaly and is caused by mutations in the gene coding for NIBRIN (NBN) which is involved in DNA damage repair. Dermal-derived fibroblasts from NBS patients were reprogrammed i...
Main Authors: | , , , , |
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Format: | Article |
Language: | English |
Published: |
Nature Publishing Group
2017-08-01
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Series: | Scientific Reports |
Online Access: | https://doi.org/10.1038/s41598-017-07905-2 |