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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Detalhes bibliográficos
Publicado no:Stem Cell Res Ther
Main Authors: 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: Artigo
Idioma:Inglês
Publicado em: BioMed Central 2019
Assuntos:
Research
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6615274/
https://ncbi.nlm.nih.gov/pubmed/31286988
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s13287-019-1305-y
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