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Inflammation Research

Published in:

01-07-2018 | Review

Inflammation, a significant player of Ataxia–Telangiectasia pathogenesis?

Authors: Majid Zaki-Dizaji, Seyed Mohammad Akrami, Gholamreza Azizi, Hassan Abolhassani, Asghar Aghamohammadi

Published in: Inflammation Research | Issue 7/2018

Abstract

Introduction

Ataxia–Telangiectasia (A-T) syndrome is an autosomal recessive neurodegenerative disorder characterized by cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, chromosome instability, radiosensitivity, and predisposition to malignancy. There is growing evidence that A–T patients suffer from pathologic inflammation that is responsible for many symptoms of this syndrome, including neurodegeneration, autoimmunity, cardiovascular disease, accelerated aging, and insulin resistance. In addition, epidemiological studies have shown A–T heterozygotes, somewhat like deficient patients, are susceptible to ionizing irradiation and have a higher risk of cancers and metabolic disorders.

Area covered

This review summarizes clinical and molecular findings of inflammation in A–T syndrome.

Conclusion

Ataxia–Telangiectasia Mutated (ATM), a master regulator of the DNA damage response is the protein known to be associated with A–T and has a complex nuclear and cytoplasmic role. Loss of ATM function may induce immune deregulation and systemic inflammation.

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Title: Inflammation, a significant player of Ataxia–Telangiectasia pathogenesis?
Authors: Majid Zaki-Dizaji
Seyed Mohammad Akrami
Gholamreza Azizi
Hassan Abolhassani
Asghar Aghamohammadi
Publication date: 01-07-2018
Publisher: Springer International Publishing
Published in: Inflammation Research / Issue 7/2018
Print ISSN: 1023-3830
Electronic ISSN: 1420-908X
DOI: https://doi.org/10.1007/s00011-018-1142-y

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