ReviewNeuromolecular medicine2006
Ataxia-telangiectasia and related diseases.
Review in Neuromolecular medicine, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
24 citing papers in PubMed, 75 citations in OpenAlex.
- Reduced FOXF1 links unrepaired DNA damage to pulmonary arterial hypertension.Nature communications · 2023Article
- Association of Oxidative Stress with Neurological Disorders.Current neuropharmacology · 2022Article
- Elevated inflammatory responses and targeted therapeutic intervention in a preclinical mouse model of ataxia-telangiectasia lung disease.Scientific reports · 2021Article
- A Dual Role of ATM in Ischemic Preconditioning and Ischemic Injury.Cellular and molecular neurobiology · 2020Article
- Neurological Manifestations of Primary Immunodeficiencies.Iranian journal of child neurology · 2018Review
- Chronic oxidative damage together with genome repair deficiency in the neurons is a double whammy for neurodegeneration: Is damage response signaling a potential therapeutic target?Mechanisms of ageing and development · 2017Review
- Polynucleotide kinase-phosphatase (PNKP) mutations and neurologic disease.Mechanisms of ageing and development · 2017Review
- Nuclear GIT2 is an ATM substrate and promotes DNA repair.Molecular and cellular biology · 2015Article
- Potential role of ATM in hepatocyte endocytosis of ApoE-deficient, ApoB48-containing lipoprotein in ApoE-deficient mice.International journal of molecular medicine · 2014Article
- Article
- Ocular involvement in primary immunodeficiency diseases.Journal of clinical immunology · 2014Review
- ISG15 deregulates autophagy in genotoxin-treated ataxia telangiectasia cells.The Journal of biological chemistry · 2013Article
- Syndromes predisposing to pediatric central nervous system tumors: lessons learned and new promises.Current neurology and neuroscience reports · 2012Article
- Primary immunodeficiency diseases associated with neurologic manifestations.Journal of clinical immunology · 2012Review
- Mre11 regulates CtIP-dependent double-strand break repair by interaction with CDK2.Nature structural & molecular biology · 2012Article
- The ATM cofactor ATMIN protects against oxidative stress and accumulation of DNA damage in the aging brain.The Journal of biological chemistry · 2010Article
- The use of neural stem cells in cancer gene therapy: predicting the path to the clinic.Current opinion in molecular therapeutics · 2010Review
- The MRN complex in double-strand break repair and telomere maintenance.FEBS letters · 2010Review
- The genesis of cerebellar interneurons and the prevention of neural DNA damage require XRCC1.Nature neuroscience · 2009Article
- DNA repair deficiency and neurological disease.Nature reviews. Neuroscience · 2009Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Appropriate cellular signaling responses to DNA damage and the ability to repair DNA are fundamental processes that are required for organismal survival. Ataxia-telangiectasia (A-T) is a rare neurodegenerative disease that results from defective DNA damage signaling. Understanding the molecular basis of A-T has provided many critical insights into the cellular response to DNA double-strand breaks (DSBs). A-T is a syndrome that shows pronounced neurodegeneration of the nervous system coincident with immune deficiency, radiosensitivity, and cancer proneness. A-T results from inactivation of the A-T mutated (ATM) kinase, a critical protein kinase that regulates the response to DNA-DSBs by selective phosphorylation of a variety of substrates. Therefore, understanding the ATM signaling program has important biological ramifications for nervous system homeostasis. Underscoring the importance of the DNA-DSBs response in the nervous system are other diseases related to A-T that also result from defects in this signaling pathway. In particular, defects in the DNA damage sensor, the Mre11-RAD50-NBS1 complex, also lead to syndromes with neurological deficits and overlapping phenotypes to A-T. Collectively, these diseases highlight the critical importance of appropriate responses to DNA-DSBs to maintain homeostasis in the nervous system.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.