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Fanconi Anemia & Oxidative Stress - Mechanistic Background & Clinical Prospects (Hardcover)
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Fanconi Anemia & Oxidative Stress - Mechanistic Background & Clinical Prospects (Hardcover)
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Fanconi Anemia (FA), a rare genetic disease featuring excess cancer
risk and chromosomal instability, has received growing interest
after the discovery that one of the defective genes in FA is
associated with high-impact diseases such as familial breast cancer
and ovary cancers. A well-documented relationship has accumulated
in several decades and up to recently, studies have linked FA with
a redox imbalance (oxidative stress, OS) in FA cells. The toxicity
mechanisms of crosslinking agents (mitomycin C and diepoxybutane)
in FA cells, along with the roles of OS-related aldehydes and
glutathione in FA and in other diseases, are critically discussed.
Also discussed is the proinflammatory state in FA phenotype.
Additionally, recent studies have discovered an impairment in the
structure and function of mitochondria in FA cell lines, while
mitochondrial dysfunction in FA patients has so far been an
unexplored field warranting ad hoc investigations. This book
provides the readers with up-to-date information and perspectives
on Fanconi anemia and oxidative stress that may prompt further
elucidation of this intriguing disease, along with working
hypotheses in clinical research and patients' management.
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