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Cells 2017, 6(3), 24; doi:10.3390/cells6030024

Induced Pluripotent Stem Cell Neuronal Models for the Study of Autophagy Pathways in Human Neurodegenerative Disease

1
Cell Biology Laboratories, School of Biochemistry, University of Bristol, Bristol BS8 1TD, UK
2
Trinity College Institute for Neuroscience, Trinity College, Dublin 2, Ireland
*
Author to whom correspondence should be addressed.
Received: 11 July 2017 / Revised: 8 August 2017 / Accepted: 9 August 2017 / Published: 11 August 2017
(This article belongs to the Special Issue Assays to Monitor Autophagy in Model Systems)
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Abstract

Human induced pluripotent stem cells (hiPSCs) are invaluable tools for research into the causes of diverse human diseases, and have enormous potential in the emerging field of regenerative medicine. Our ability to reprogramme patient cells to become hiPSCs, and to subsequently direct their differentiation towards those classes of neurons that are vulnerable to stress, is revealing how genetic mutations cause changes at the molecular level that drive the complex pathogeneses of human neurodegenerative diseases. Autophagy dysregulation is considered to be a major contributor in neural decline during the onset and progression of many human neurodegenerative diseases, meaning that a better understanding of the control of non-selective and selective autophagy pathways (including mitophagy) in disease-affected classes of neurons is needed. To achieve this, it is essential that the methodologies commonly used to study autophagy regulation under basal and stressed conditions in standard cell-line models are accurately applied when using hiPSC-derived neuronal cultures. Here, we discuss the roles and control of autophagy in human stem cells, and how autophagy contributes to neural differentiation in vitro. We also describe how autophagy-monitoring tools can be applied to hiPSC-derived neurons for the study of human neurodegenerative disease in vitro. View Full-Text
Keywords: autophagy; mitophagy; autophagic flux; stem cells; pluripotency; hiPSC; neurons autophagy; mitophagy; autophagic flux; stem cells; pluripotency; hiPSC; neurons
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MDPI and ACS Style

Jiménez-Moreno, N.; Stathakos, P.; Caldwell, M.A.; Lane, J.D. Induced Pluripotent Stem Cell Neuronal Models for the Study of Autophagy Pathways in Human Neurodegenerative Disease. Cells 2017, 6, 24.

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