TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes

Mitochondrial trifunctional protein deficiency, due to mutations in hydratase subunit A (HADHA), results in sudden infant death syndrome with no cure. To reveal the disease etiology, we generated stem cell-derived cardiomyocytes from HADHA-deficient hiPSCs and accelerated their maturation via an eng...

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Detalles Bibliográficos
Autores principales: Miklas, Jason W., Clark, Elisa, Levy, Shiri, Detraux, Damien, Leonard, Andrea, Beussman, Kevin, Showalter, Megan R., Smith, Alec T., Hofsteen, Peter, Yang, Xiulan, Macadangdang, Jesse, Manninen, Tuula, Raftery, Daniel, Madan, Anup, Suomalainen, Anu, Kim, Deok-Ho, Murry, Charles E., Fiehn, Oliver, Sniadecki, Nathan J., Wang, Yuliang, Ruohola-Baker, Hannele
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6789043/
https://www.ncbi.nlm.nih.gov/pubmed/31604922
http://dx.doi.org/10.1038/s41467-019-12482-1

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6789043/
https://www.ncbi.nlm.nih.gov/pubmed/31604922
http://dx.doi.org/10.1038/s41467-019-12482-1

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