2017
DOI: 10.18632/aging.101247
View full text |Buy / Rent full text
|
Sign up to set email alerts
|

Abstract: We sought to identify the impacts of Friedreich's ataxia (FRDA) on cardiomyocytes. FRDA is an autosomal recessive degenerative condition with neuronal and non-neuronal manifestations, the latter including progressive cardiomyopathy of the left ventricle, the leading cause of death in FRDA. Little is known about the cellular pathogenesis of FRDA in cardiomyocytes. Induced pluripotent stem cells (iPSCs) were derived from three FRDA individuals with characterized GAA repeats. The cells were differentiated into ca… Show more

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
2
2
1

Citation Types

0
17
0

Year Published

2017
2017
2018
2018

Publication Types

Select...
5

Relationship

3
2

Authors

Journals

citations
Cited by 23 publications
(17 citation statements)
references
References 42 publications
(49 reference statements)
0
17
0
Order By: Relevance
“…Fibroblasts were reprogrammed to hiPSCs with nonintegrating episomal vectors containing six reprogramming factors (OCT4, SOX2, KLF4, L-MYC, LIN28, and shRNA for p53) (60). More detailed information is presented in SI Appendix, SI Methods.…”
Section: Discussionmentioning
confidence: 99%
“…Fibroblasts were reprogrammed to hiPSCs with nonintegrating episomal vectors containing six reprogramming factors (OCT4, SOX2, KLF4, L-MYC, LIN28, and shRNA for p53) (60). More detailed information is presented in SI Appendix, SI Methods.…”
Section: Discussionmentioning
confidence: 99%
“…The authors obtained mutation-free iPSCs and in these cybrid corrected iPSC-derived retinal ganglion cell, the cell death phenotype was res cued. The usefulness of the iPSC model is also offered by the work of Crombie et al [ 32 ], in which, Friedreich's ataxia (FRDA) iPSCs have been differentiated into cardiomyocytes to be used for future high throughput compound screening. In line with these iPSCs models, we suggest that our aging model provides an opportunity to investigate stem cell properties and the aging biology of stem cells.…”
Section: Discussionmentioning
confidence: 99%
“…Extracellular field potentials were recorded at baseline and 2 min after addition of drugs. Data were analysed offline with MC Rack version 4.3.5 software for beating rate, RR interval and extracellular field potential duration (FPD) as previously described 35 , 36 . FPD measurements were normalized (corrected FPD, cFPD) with the Bazzet correction formula: cFPD = FPD/√(RR interval).…”
Section: Methodsmentioning
confidence: 99%