نتایج جستجو برای: ataxia telangiectasia

تعداد نتایج: 20288  

Journal: :Archives of Disease in Childhood 1962

Journal: :Redox report : communications in free radical research 2003
Dianne J Watters

Ataxia telangiectasia is one of a group of recessive hereditary genomic instability disorders and is characterized by progressive neurodegeneration, immunodeficiency and cancer susceptibility. Heterozygotes for the mutated gene are more susceptible to cancer and to ischaemic heart disease. The affected gene, ATM (ataxia telangiectasia mutated), has been cloned and codes for a protein kinase (AT...

Journal: :Clinical cancer research : an official journal of the American Association for Cancer Research 2008
Zhibin Hu Hongliang Liu Haifeng Wang Ruifen Miao Weiwei Sun Guangfu Jin Ying Wang Hongxia Ma Li Jin Qingyi Wei Daru Lu Wei Huang Hongbing Shen

PURPOSE DNA damage checkpoints are initiated by its sensor proteins of the phosphoinositide-3-kinase-related protein kinase family, including ataxia-telangiectasia mutated, ataxia-telangiectasia and Rad3-related, and DNA-dependent protein kinase catalytic subunit (DNA-PKcs). We hypothesized that polymorphisms in these genes may alter the regulation of DNA repair and the risk of lung cancer. E...

Journal: :Movement disorders : official journal of the Movement Disorder Society 2014
Ishani Sahama Kate Sinclair Simona Fiori Kerstin Pannek Martin Lavin Stephen Rose

Magnetic resonance imaging (MRI) research in identifying altered brain structure and function in ataxia-telangiectasia, an autosomal recessive neurodegenerative disorder, is limited. Diffusion-weighted MRI were obtained from 11 ataxia telangiectasia patients (age range, 7-22 years; mean, 12 years) and 11 typically developing age-matched participants (age range, 8-23 years; mean, 13 years). Gray...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2011
Leo E Otterbein Andreas Hedblom Clair Harris Eva Csizmadia David Gallo Barbara Wegiel

Stability and repair of DNA is of principal importance in cell survival. Heme oxygenase-1 (HO-1; Hmox1) is critical in maintaining cellular homeostasis, in large part through its ability to generate CO, but neither molecule has been studied in the setting of DNA damage. Naïve Hmox1(-/-) mice exhibit excessive tissue levels of γ-histone H2A, whereas administration of genotoxic stressors or irrad...

Journal: :The European respiratory journal 2016
Michiel H C Slaats Wouter Hoefsloot Cecile Magis-Escurra Martin J Boeree Melanie Wattenberg Saskia Kuipers Jakko van Ingen

women with breast cancer. Oncologist 2014; 19: 1216–1226. 8 Katayama N, Sato S, Katsui K, et al. Analysis of factors associated with radiation-induced bronchiolitis obliterans organizing pneumonia syndrome after breast-conserving therapy. Int J Radiat Oncol Biol Phys 2009; 73: 1049–1054. 9 Bhatt JM, Bush A, van Gerven M, et al. A statement on the multidisciplinary respiratory management of atax...

Ataxia-Telangiectasia (AT) is a rare human neurodegenerative autosomal recessive multisystem disease that is characterized by a wide range of features including, progressive cerebellar ataxia with onset during infancy, occulocutaneous telangiectasia, susceptibility to neoplasia, occulomotor disturbances, chromosomal instability and growth and developmental abnormalities. Mitochondrial DNA (mtDN...

2015
Ishani Sahama Kate Sinclair Simona Fiori James Doecke Kerstin Pannek Lee Reid Martin Lavin Stephen Rose

BACKGROUND Our understanding of the effect of ataxia-telangiectasia mutated gene mutations on brain structure and function is limited. In this study, white matter motor pathway integrity was investigated in ataxia telangiectasia patients using diffusion MRI and probabilistic tractography. METHODS Diffusion MRI were obtained from 12 patients (age range: 7-22 years, mean: 12 years) and 12 typic...

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