Chapter category: DNA Surveillance and Repair

Cell Signaling in Ataxia Telangiectasia

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:
Tetsuo Nakajima

[+] view image

Ataxia-telangiectasia (AT) is a disease with pleiotropic defects that include hypersensitivity to ionizing radiation, immunodeficiency and increased cancer risk and Ataxia Telangiectasia Mutated (ATM) gene is responsible for AT. Particularly since AT patients are strongly radiosensitive, the relationship between ATM and DNA damage has been studied thoroughly. ATM recognizes the DNA double-strand breaks produced by DNA damaging agents such as ionizing radiation and subsequently controls cell fate by DNA repair, cell cycle regulation and apoptosis. Cell signaling cascades involving ATM are diverse and in terms of radiation-induced apoptosis, numerous participants and their roles have been uncovered in recent years. Here, ATM-related signaling cascades are reviewed, focusing on radiation-induced apoptosis. Relationships with other signaling cascades, such as oxidative stress-related and insulin-related signaling are also discussed.

Tetsuo Nakajima
Research Center for Radiation Protection, National Institute of Radiological Sciences, Chiba, Japan

» Access chapter for $19

Additional chapters from this book:

Oncogenesis in Ataxia Telangectasia: Roles of Atm, p53, Nf‑kb and DDE recombination pathogenesis

David H. Dreyfus

The mechanistic basis of ATM (Ataxia Telangectasis Mutated) protein interactions with DNA hairpin and related double stranded DNA breaks, generated through V(D)J recombination, has been the subject of...

Cell Signaling in Ataxia Telangiectasia

Tetsuo Nakajima

Protein‑Protein Interactions in Ataxia Telangiectasia

Steven M. Shell and Yue Zou

Ataxia telangiectasia (AT) is an early‑onset genetic disorder characterized by progressive neurondegeneration, chromosome instability and an extreme sensitivity to DNA damaging agents such as io...

Ataxia Telangiectasia and its Overlap with Nijmegen Breakage Syndrome and Ataxia‑Like Disorders

Lindsay G. Ball and Wei Xiao

Ataxia Telangiectasia (AT), Nijmegen Breakage Syndrome (NBS) and AT‑Like Disorder (ATLD) are three closely related human diseases. AT, NBS and ATLD share several prominent cellular phenotypes in...

DNA Damage and Repair in Ataxia Telangiectasia

Melissa M. Adams and Phillip Carpenter

Defects in the gene Ataxia Telangiectasia Mutated (ATM) are responsible for the development of Ataxia Telangiectasia (AT), an incurable cancer‑prone disease that is accompanied by a pleiotropic ...

Cell Cycle Defects and Apoptosis in Ataxia telangiectasia

Deborah Wilsker and Fred Bunz

ATM is a key regulator of both cell cycle checkpoints and apoptosis. Cells lacking ATM are defective in critical responses to damaged DNA and particularly to the double strand DNA breaks caused by ion...

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

Luitpold V. Distel

Ataxia telangiectasia (AT) is a syndrome with multiple symptoms, of them one is the frequent and early onset of cancer. The disease is triggered by a mutation in the ATM‑gene. The gene and its e...

Chapter category: DNA Surveillance and Repair

Cell Signaling in Ataxia Telangiectasia

Additional chapters from this book:

SIGN IN