A Patient-Specific Stem Cell Model to Investigate the Neurological Phenotype Observed in Ataxia-Telangiectasia
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Wali, Gautam
Perry, Chris
Lavin, Martin F
Feron, Francois
Mackay-Sim, Alan
Sutharsan, Ratneswary
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Kozlov, SV
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Abstract
The molecular pathogenesis of ataxia-telangiectasia (A-T) is not yet fully understood, and a versatile cellular model is required for in vitro studies. The occurrence of continuous neurogenesis and easy access make the multipotent adult stem cells from the olfactory mucosa within the nasal cavity a potential cellular model. We describe an efficient method to establish neuron-like cells from olfactory mucosa biopsies derived from A-T patients for the purpose of studying the cellular and molecular aspects of this debilitating disease.
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ATM Kinase: Methods and Protocols
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Other chemical sciences
Biochemistry and cell biology
Genetics not elsewhere classified