期刊
PEDIATRIC BLOOD & CANCER
卷 62, 期 7, 页码 1137-1143出版社
WILEY
DOI: 10.1002/pbc.25452
关键词
bioactivation; crosslinkers; diepoxybutane; FANC proteins; Fanconi anemia; glutathione; melphalan; mitomycin C; oxidative stress
The commonly accepted definition of Fanconi anemia (FA) relying on DNA repair deficiency is submitted to a critical review starting from the early reports pointing to mitomycin C bioactivation and to the toxicity mechanisms of diepoxybutane and a group of nitrogen mustards causing DNA crosslinks in FA cells. A critical analysis of the literature prompts revisiting the FA phenotype and crosslinker sensitivity in terms of an oxidative stress (OS) background, redox-related anomalies of FA (FANC) proteins, and mitochondrial dysfunction. This re-appraisal of FA basic defect might lead to innovative approaches both in elucidating FA phenotypes and in clinical management. Pediatr Blood Cancer 2015;62:1137-1143. (c) 2015 Wiley Periodicals, Inc.
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