scholarly journals Nitration of microtubules blocks axonal mitochondrial transport in a human pluripotent stem cell model of Parkinson's disease

2018 ◽  
Vol 32 (10) ◽  
pp. 5350-5364 ◽  
Author(s):  
Morgan G. Stykel ◽  
Kayla Humphries ◽  
Mathew P. Kirby ◽  
Chris Czaniecki ◽  
Tinya Wang ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Stem Cell ◽  
Pluripotent Stem Cell ◽  
Cell Model ◽  
Human Pluripotent Stem Cell ◽  
Mitochondrial Transport ◽  
Stem Cell Model

scholarly journals Using Human Pluripotent Stem Cell–Derived Dopaminergic Neurons to Evaluate Candidate Parkinson’s Disease Therapeutic Agents in MPP+ and Rotenone Models

2013 ◽  
Vol 18 (5) ◽  
pp. 522-533 ◽  
Author(s):  
Jun Peng ◽  
Qiuyue Liu ◽  
Mahendra S. Rao ◽  
Xianmin Zeng
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Stem Cell ◽  
Dopaminergic Neurons ◽  
Pluripotent Stem Cell ◽  
Neuroprotective Effect ◽  
Neuronal Cell ◽  
Rapid Screening ◽  
Patient Specific ◽  
Human Pluripotent Stem Cell

To begin to develop a high-throughput assay system to evaluate potential small-molecule therapy for Parkinson’s disease (PD), we have performed a low-throughput assay with a small number of compounds using human pluripotent stem cell–derived dopaminergic neurons. We first evaluated the role of 44 compounds known to work in rodent systems in a 1-methyl-4-phenylpyridinium (MPP+) assay in a 96-well format using the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide assay as a readout for neuroprotection. Glial cell–derived neurotrophic factor was used as a positive control because of its well-documented neuroprotective effect on dopaminergic neurons, and two concentrations of each drug were tested. Of 44 compounds screened, 16 showed a neuroprotective effect at one or both dosages tested. A dose-response curve of a subset of the 16 positives was established in the MPP+ model. In addition, we validated neuroprotective effects of these compounds in a rotenone-induced dopaminergic neuronal cell death, another established model for PD. Our human primary dopaminergic neuron-based assays provide a platform for rapid screening and/or validation of potential neuroprotective agents in PD treatment using patient-specific cells and show the importance of using human cells for such assays.

scholarly journals A human pluripotent stem cell model for the analysis of metabolic dysfunction in hepatic steatosis

iScience ◽  
2021 ◽  
Vol 24 (1) ◽  
pp. 101931
Author(s):  
Matthew C. Sinton ◽  
Jose Meseguer-Ripolles ◽  
Baltasar Lucendo-Villarin ◽  
Sara Wernig-Zorc ◽  
John P. Thomson ◽  
...  
Keyword(s):  
Stem Cell ◽  
Hepatic Steatosis ◽  
Pluripotent Stem Cell ◽  
Cell Model ◽  
Metabolic Dysfunction ◽  
Human Pluripotent Stem Cell ◽  
Stem Cell Model

scholarly journals Molecular analyses of neurogenic defects in a human pluripotent stem cell model of fragile X syndrome

Brain ◽  
2017 ◽  
pp. aww357 ◽  
Author(s):  
Michael J. Boland ◽  
Kristopher L. Nazor ◽  
Ha T. Tran ◽  
Attila Szücs ◽  
Candace L. Lynch ◽  
...  
Keyword(s):  
Stem Cell ◽  
Fragile X Syndrome ◽  
Pluripotent Stem Cell ◽  
Cell Model ◽  
Fragile X ◽  
Human Pluripotent Stem Cell ◽  
Molecular Analyses ◽  
Stem Cell Model

scholarly journals A Human Pluripotent Stem Cell Model of Facioscapulohumeral Muscular Dystrophy‐Affected Skeletal Muscles

2016 ◽  
Vol 5 (9) ◽  
pp. 1145-1161 ◽  
Author(s):  
Leslie Caron ◽  
Devaki Kher ◽  
Kian Leong Lee ◽  
Robert McKernan ◽  
Biljana Dumevska ◽  
...  
Keyword(s):  
Stem Cell ◽  
Muscular Dystrophy ◽  
Skeletal Muscles ◽  
Pluripotent Stem Cell ◽  
Cell Model ◽  
Facioscapulohumeral Muscular Dystrophy ◽  
Human Pluripotent Stem Cell ◽  
Stem Cell Model
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