scholarly journals Corrigendum to ‘Efficient attenuation of Friedreich's ataxia (FRDA) cardiomyopathy by modulation of iron homeostasis-human induced pluripotent stem cell (hiPSC) as a drug screening platform for FRDA’ [Int J Cardiol 203 (2016) 964–971]

2016 ◽  
Vol 207 ◽  
pp. 393 ◽  
Author(s):  
Y.K. Lee ◽  
Y.M. Lau ◽  
K.M. Ng ◽  
W.H. Lai ◽  
S.L. Ho ◽  
...  
Aging ◽  
2017 ◽  
Vol 9 (5) ◽  
pp. 1440-1452 ◽  
Author(s):  
Duncan E. Crombie ◽  
Claire L. Curl ◽  
Antonia JA Raaijmakers ◽  
Priyadharshini Sivakumaran ◽  
Tejal Kulkarni ◽  
...  

Neurology ◽  
2012 ◽  
Vol 78 (Meeting Abstracts 1) ◽  
pp. P05.022-P05.022
Author(s):  
M. Pandolfo ◽  
S. Chintawar ◽  
M. Wattenhofer-Donze ◽  
H. Puccio

Neurology ◽  
2012 ◽  
Vol 78 (Meeting Abstracts 1) ◽  
pp. IN8-1.007-IN8-1.007
Author(s):  
M. Pandolfo ◽  
S. Chintawar ◽  
M. Wattenhofer-Donze ◽  
H. Puccio

2020 ◽  
Vol 10 (1) ◽  
pp. 68-82
Author(s):  
Marvin G. Gunawan ◽  
Sarabjit S. Sangha ◽  
Sanam Shafaattalab ◽  
Eric Lin ◽  
Danielle A. Heims‐Waldron ◽  
...  

2020 ◽  
Author(s):  
Marvin G. Gunawan ◽  
Sarabjit S. Sangha ◽  
Sanam Shafaattalab ◽  
Eric Lin ◽  
Danielle A. Heims-Waldron ◽  
...  

AbstractCurrent drug development efforts for the treatment of atrial fibrillation (AF) are hampered by the fact that many preclinical models have been unsuccessful in reproducing human cardiac atrial physiology and its response to medications. In this study, we demonstrated an approach using human induced pluripotent stem cell-derived atrial and ventricular cardiomyocytes (hiPSC-aCMs and hiPSC-vCMs, respectively) coupled with a sophisticated optical mapping system for drug screening of atrial-selective compounds in vitro.We optimized differentiation of hiPSC-aCMs by modulating the WNT and retinoid signalling pathways. Characterization of the transcriptome and proteome revealed that retinoic acid pushes the differentiation process into the atrial lineage and generated hiPSC-aCMs. Functional characterization using optical mapping showed that hiPSC-aCMs have shorter action potential durations and faster Ca2+ handling dynamics compared to hiPSC-vCMs. Furthermore, pharmacological investigation of hiPSC-aCMs captured atrial-selective effects by displaying greater sensitivity to atrial-selective compounds 4-aminopyridine, AVE0118, UCL1684, and vernakalant when compared to hiPSC-vCMs.These results established that a model system incorporating hiPSC-aCMs combined with optical mapping is well-suited for pre-clinical drug screening of novel and targeted atrial selective compounds.


2016 ◽  
Vol 2016 ◽  
pp. 1-15 ◽  
Author(s):  
Aoife Gowran ◽  
Marco Rasponi ◽  
Roberta Visone ◽  
Patrizia Nigro ◽  
Gianluca L. Perrucci ◽  
...  

A mere 9 years have passed since the revolutionary report describing the derivation of induced pluripotent stem cells from human fibroblasts and the first in-patient translational use of cells obtained from these stem cells has already been achieved. From the perspectives of clinicians and researchers alike, the promise of induced pluripotent stem cells is alluring if somewhat beguiling. It is now evident that this technology is nascent and many areas for refinement have been identified and need to be considered before induced pluripotent stem cells can be routinely used to stratify, treat and cure patients, and to faithfully model diseases for drug screening purposes. This review specifically addresses the pioneering approaches to improve induced pluripotent stem cell based models of nonischaemic cardiomyopathy.


2020 ◽  
Author(s):  
Valentin Parat ◽  
Brigitte Onteniente ◽  
Julien Maruotti

AbstractIn this study, we describe a simple and straight-forward assay using induced pluripotent stem cell derived melanocytes and high-throughput flow cytometry, to screen and identify pigment regulating agents. The assays is based on the correlation between forward light-scatter characteristics and melanin content, with pigmented cells displaying high light absorption/low forward light-scatter, while the opposite is true for lowly pigmented melanocytes, as a result of genetic background or chemical treatments. Orthogonal validation is then performed by regular melanin quantification. Such approach was validated using a set of 80 small molecules, and yielded a confirmed hit. The assay described in this study may prove a useful tool to identify modulators of melanogenesis in human melanocytes.


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