Nature Communications (Oct 2019)
TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes
- Jason W. Miklas,
- Elisa Clark,
- Shiri Levy,
- Damien Detraux,
- Andrea Leonard,
- Kevin Beussman,
- Megan R. Showalter,
- Alec T. Smith,
- Peter Hofsteen,
- Xiulan Yang,
- Jesse Macadangdang,
- Tuula Manninen,
- Daniel Raftery,
- Anup Madan,
- Anu Suomalainen,
- Deok-Ho Kim,
- Charles E. Murry,
- Oliver Fiehn,
- Nathan J. Sniadecki,
- Yuliang Wang,
- Hannele Ruohola-Baker
Affiliations
- Jason W. Miklas
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Elisa Clark
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Shiri Levy
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Damien Detraux
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Andrea Leonard
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Kevin Beussman
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Megan R. Showalter
- NIH West Coast Metabolomics Center, University of California Davis
- Alec T. Smith
- Department of Bioengineering, University of Washington
- Peter Hofsteen
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Xiulan Yang
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Jesse Macadangdang
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Tuula Manninen
- Helsinki University Hospital
- Daniel Raftery
- Department of Anesthesiology and Pain Medicine, Mitochondria and Metabolism Center, University of Washington
- Anup Madan
- Covance Genomics Laboratory
- Anu Suomalainen
- Helsinki University Hospital
- Deok-Ho Kim
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Charles E. Murry
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Oliver Fiehn
- NIH West Coast Metabolomics Center, University of California Davis
- Nathan J. Sniadecki
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Yuliang Wang
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- Hannele Ruohola-Baker
- Institute for Stem Cell and Regenerative Medicine, University of Washington, School of Medicine
- DOI
- https://doi.org/10.1038/s41467-019-12482-1
- Journal volume & issue
-
Vol. 10,
no. 1
pp. 1 – 21
Abstract
Mutations in the gene HADHA result in mitochondrial tri-functional protein (MTP) deficiency and can result in sudden infant death syndrome for which there is no treatment. Here the authors show that the MTP deficient pathology in human cardiomyocytes leads to an abnormal cardiolipin pattern and suggests that cardiolipin affecting compounds may serve as a potential therapy.
