نتایج جستجو برای: ataxia telangiectasia

تعداد نتایج: 20288  

Journal: :Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 1983

Journal: :Cancer research 2000
M Gatei S P Scott I Filippovitch N Soronika M F Lavin B Weber K K Khanna

The human genetic disorder ataxia-telangiectasia is characterized by immunodeficiency, progressive cerebellar ataxia, radiosensitivity, cell cycle checkpoint defects, and cancer predisposition. The gene product [ataxia-telangiectasia mutation (ATM)] mutated in this syndrome is a component of the DNA damage detection pathway. Loss of ATM function in human and mouse cells causes defects in DNA re...

Journal: :Arquivos de Neuro-Psiquiatria 1979

Journal: :International Journal of Case Reports and Images 2012

2010
Laura Folgori Alessia Scarselli Giulia Angelino Francesca Ferrari Antonio Antoccia Luciana Chessa Andrea Finocchi

Ataxia-telangiectasia (A-T) is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM) gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children....

2017
Erika S. Dahl Katherine M. Aird

The ataxia-telangiectasia mutated (ATM) protein kinase has been extensively studied for its role in the DNA damage response and its association with the disease ataxia telangiectasia. There is increasing evidence that ATM also plays an important role in other cellular processes, including carbon metabolism. Carbon metabolism is highly dysregulated in cancer due to the increased need for cellula...

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