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Molecular Mechanisms of Fanconi Anemia pp 82–91Cite as

Fanconi Anaemia and Oxidative Stress

Cellular and Clinical Phenotypes

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Abstract

The cellular and clinical phenotypes of Fanconi Anaemia (FA) have been associated with a set of redox abnormalities using evidence arising from in vitro, in vivo and molecular studies. The available information points to: (i) the influence of oxygen and antioxidants in chromosomal instability and in apoptosis; (ii) the redox-related toxicity mechanisms of agents (commonly termed “crosslinkers”) triggering excess sensitivity of FA cells; (iii) a set of abnormalities in redox biomarkers detected in body fluids and blood cells from FA patients; (iv) a number of clinical features related to a chronic pro-oxidant state, and (v) the involvement of redox pathways in the functions and structures of at least three proteins encoded by FA genes (FANCA, FANCC and FANCG). Oxidative stress may thus be envisaged as an important phenomenon in FA accounting for most of the findings observed in FA’s clinical phenotype. This information ought to prompt clinical studies that might unveil new avenues in FA research, such as the prospect of controlled chemoprevention trials aimed at counteracting the FA-associated pro-oxidant state and ameliorating FA’s clinical course.

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  1. Innovation and Technology Transfer in Oncology and Life Sciences, Centre for Research, I-83013, Mercogliano (AV), Italy

    Giovanni Pagano

  2. School of Biomedical and Natural Sciences, Nottingham Trent University, Nottingham, England, UK

    Shamim I. Ahmad

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Pagano, G., Ahmad, S.I. (2006). Fanconi Anaemia and Oxidative Stress. In: Molecular Mechanisms of Fanconi Anemia. Medical Intelligence Unit. Springer, Boston, MA. https://doi.org/10.1007/0-387-33776-8_9

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