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A Patient-Specific Stem Cell Model to Investigate the Neurological Phenotype Observed in Ataxia-Telangiectasia.
Stewart, Romal; Wali, Gautam; Perry, Chris; Lavin, Martin F; Féron, Francois; Mackay-Sim, Alan; Sutharsan, Ratneswary.
Affiliation
  • Stewart R; University of Queensland Centre for Clinical Research, Brisbane, QLD, 4006, Australia.
  • Wali G; Eskitis Institute for Drug Discovery, Griffith University, Nathan, QLD, 4111, Australia.
  • Perry C; Department of Otolaryngology Head and Neck Surgery, Princess Alexandra Hospital, Woolloongabba, Brisbane, QLD, 4012, Australia.
  • Lavin MF; University of Queensland Centre for Clinical Research (UQCCR), University of Queensland, Herston, Brisbane, QLD, 4006, Australia.
  • Féron F; Aix Marseille Université, CNRS, NICN, UMR7259, 13344, Marseille, France.
  • Mackay-Sim A; APHM, Centre d'Investigations Cliniques en Biothérapie, CIC-BT 510, Marseille, France.
  • Sutharsan R; Eskitis Institute for Drug Discovery, Griffith University, Nathan, QLD, 4111, Australia.
Methods Mol Biol ; 1599: 391-400, 2017.
Article in En | MEDLINE | ID: mdl-28477134
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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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Stem Cells / Ataxia Telangiectasia / Ataxia Telangiectasia Mutated Proteins Limits: Humans Language: En Journal: Methods Mol Biol Journal subject: BIOLOGIA MOLECULAR Year: 2017 Document type: Article Affiliation country: Australia
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Stem Cells / Ataxia Telangiectasia / Ataxia Telangiectasia Mutated Proteins Limits: Humans Language: En Journal: Methods Mol Biol Journal subject: BIOLOGIA MOLECULAR Year: 2017 Document type: Article Affiliation country: Australia