Accumulation of cytoplasmic DNA due to ATM deficiency activates the microglial viral response system with neurotoxic consequences

X Song, F Ma, K Herrup - Journal of Neuroscience, 2019 - Soc Neuroscience
ATM (ataxia-telangiectasia mutated) is a PI3K-like kinase best known for its role in the DNA
damage response (DDR), especially after double-strand breaks. Mutations in the ATM gene …

Persistent DNA damage associated with ATM kinase deficiency promotes microglial dysfunction

J Bourseguin, W Cheng, E Talbot, L Hardy… - Nucleic acids …, 2022 - academic.oup.com
The autosomal recessive genome instability disorder Ataxia–telangiectasia, caused by
mutations in ATM kinase, is characterized by the progressive loss of cerebellar neurons. We …

Nuclear ataxia-telangiectasia mutated (ATM) mediates the cellular response to DNA double strand breaks in human neuron-like cells

S Biton, I Dar, L Mittelman, Y Pereg, A Barzilai… - Journal of Biological …, 2006 - ASBMB
The protein kinase ATM (ataxia-telangiectasia mutated) activates the cellular response to
double strand breaks (DSBs), a highly cytotoxic DNA lesion. ATM is activated by DSBs and …

ATM kinase inhibition in glial cells activates the innate immune response and causes neurodegeneration in Drosophila

AJ Petersen, SA Rimkus… - Proceedings of the …, 2012 - National Acad Sciences
To investigate the mechanistic basis for central nervous system (CNS) neurodegeneration in
the disease ataxia–telangiectasia (AT), we analyzed flies mutant for the causative gene AT …

Neurodegeneration in ataxia‐telangiectasia: Multiple roles of ATM kinase in cellular homeostasis

KR Choy, DJ Watters - Developmental Dynamics, 2018 - Wiley Online Library
Ataxia‐telangiectasia (A‐T) is characterized by neuronal degeneration, cancer, diabetes,
immune deficiency, and increased sensitivity to ionizing radiation. A‐T is attributed to the …

DNA damage primes the type I interferon system via the cytosolic DNA sensor STING to promote anti-microbial innate immunity

A Härtlova, SF Erttmann, FAM Raffi, AM Schmalz… - Immunity, 2015 - cell.com
Dysfunction in Ataxia-telangiectasia mutated (ATM), a central component of the DNA repair
machinery, results in Ataxia Telangiectasia (AT), a cancer-prone disease with a variety of …

Ataxia-telangiectasia and related diseases

PO Frappart, PJ McKinnon - Neuromolecular medicine, 2006 - Springer
Appropriate cellular signaling responses to DNA damage and the ability to repair DNA are
fundamental processes that are required for organismal survival. Ataxia-telangiectasia (AT) …

Cytoplasmic ATM in neurons modulates synaptic function

J Li, YR Han, MR Plummer, K Herrup - Current Biology, 2009 - cell.com
ATM is a PI 3-kinase involved in DNA double-strand break repair. ATM deficiency leads to
ataxia-telangiectasia (AT), a syndrome of cancer susceptibility, hypersensitivity to ionizing …

ATM and the DNA damage response: Workshop on Ataxia‐Telangiectasia and Related Syndromes

MF Lavin, D Delia, L Chessa - EMBO reports, 2006 - embopress.org
This workshop on ataxia-telangiectasia marked the tenth anniversary of the discovery of the
gene that is defective in this syndrome—that is, ataxia-telangiectasia mutated (ATM; Savitsky …

The role of ATM and DNA damage in neurons: upstream and downstream connections

K Herrup, J Li, J Chen - DNA repair, 2013 - Elsevier
ATM (ataxia-telangiectasia mutated) is a large protein kinase whose best-known function is
as a participant in the process of DNA damage repair, specifically lesions that result in …