Correlation between frataxin expression and contractility revealed by in vitro Friedreich’s ataxia cardiac tissue models engineered from human pluripotent stem cells

BACKGROUND: Friedreich’s ataxia (FRDA) is an autosomal recessive disease caused by a non-coding mutation in the first intron of the frataxin (FXN) gene that suppresses its expression. Compensatory hypertrophic cardiomyopathy, dilated cardiomyopathy, and conduction system abnormalities in FRDA lead t...

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Detalles Bibliográficos
Autores principales: Wong, Andy On-Tik, Wong, Gabriel, Shen, Michael, Chow, Maggie Zi-Ying, Tse, Wan Wai, Gurung, Bimal, Mak, Suet Yee, Lieu, Deborah K., Costa, Kevin D., Chan, Camie W., Martelli, Alain, Nabhan, Joseph F., Li, Ronald A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6615274/
https://www.ncbi.nlm.nih.gov/pubmed/31286988
http://dx.doi.org/10.1186/s13287-019-1305-y

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6615274/
https://www.ncbi.nlm.nih.gov/pubmed/31286988
http://dx.doi.org/10.1186/s13287-019-1305-y

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