Literature DB >> 28477134

A Patient-Specific Stem Cell Model to Investigate the Neurological Phenotype Observed in Ataxia-Telangiectasia.

Romal Stewart1, Gautam Wali2, Chris Perry3, Martin F Lavin4, Francois Féron5,6, Alan Mackay-Sim2, Ratneswary Sutharsan7,8.   

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.

Entities:  

Keywords:  Ataxia-telangiectasia; Neuronal differentiation; Olfactory mucosa; Stem cells

Mesh:

Substances:

Year:  2017        PMID: 28477134     DOI: 10.1007/978-1-4939-6955-5_28

Source DB:  PubMed          Journal:  Methods Mol Biol        ISSN: 1064-3745


  2 in total

1.  Expanding the spectrum of PEX16 mutations and novel insights into disease mechanisms.

Authors:  Kishore R Kumar; Gautam Wali; Ryan L Davis; Amali C Mallawaarachchi; Elizabeth E Palmer; Velimir Gayevskiy; Andre E Minoche; David Veivers; Marcel E Dinger; Alan Mackay-Sim; Mark J Cowley; Carolyn M Sue
Journal:  Mol Genet Metab Rep       Date:  2018-07-20

Review 2.  Analyzing Olfactory Neuron Precursors Non-Invasively Isolated through NADH FLIM as a Potential Tool to Study Oxidative Stress in Alzheimer's Disease.

Authors:  Laura Gómez-Virgilio; Alejandro Luarte; Daniela P Ponce; Bárbara A Bruna; María I Behrens
Journal:  Int J Mol Sci       Date:  2021-06-12       Impact factor: 5.923

  2 in total

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