Chapter category:

Ataxia Telangiectasia: An Oxidative Stress‑Related Disease

This chapter appears in the following book:

Molecular Mechanisms
of Ataxia Telangiectasia

Edited by: Shamim I. Ahmad
ISBN: TBA
» Get more information about this book at landesbioscience.com «

Chapter authors:
Giovanni Pagano, Paolo Degan and Giuseppe Castello

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Multi‑factorial information is available on Ataxia Telangiectasia (AT) phenotype associated with oxidative stress. First, the protein encoded by the AT gene (ATM) is involved in both the regulation of responses to double strand DNA breaks and of response to oxidative stress. Moreover, an established collection of literature points to the roles of oxidative stress in AT that can be summarized as follows: (i) increased sensitivity of AT cells to DNA damaging agents via free radical mechanisms; (ii) increased sensitivity of AT cells to inflammatory cells and to ROS; (iii) protective effects of synthetic antioxidants in Atm knock‑out mice; (iv) regulation of oxidative stress response in cell proliferation vs apoptosis, (v) abnormal levels of oxidative stress parameters in body fluids and blood cells from AT patients and (vi) impairment of mitochondrial functions and structure. Oxidative stress may thus be envisaged as a major phenomenon in AT’s clinical phenotype.

Giovanni Pagano
Ahmad, Shamim

Paolo Degan

Giuseppe Castello

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Additional chapters from this book:

Oncogenesis in Ataxia Telangectasia: Roles of ATM, p53, NF‑kB and DDE Recombination Pathogenesis

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Ataxia Telangiectasia: An Oxidative Stress‑Related Disease

Giovanni Pagano, Paolo Degan and Giuseppe Castello

Multi‑factorial information is available on Ataxia Telangiectasia (AT) phenotype associated with oxidative stress. First, the protein encoded by the AT gene (ATM) is involved in both the regulat...

Chromosomal Instability in Ataxia Telangiectasia

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Chapter category:

Ataxia Telangiectasia: An Oxidative Stress‑Related Disease

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