Elucidating the effect of drug-induced mitochondrial dysfunction on insulin signaling and glucose handling in skeletal muscle cell line (C2C12) in vitro. [PDF]
Kuretu A +3 more
europepmc +1 more source
Author Correction: Upregulation of the AMPK-FOXO1-PDK4 pathway is a primary mechanism of pyruvate dehydrogenase activity reduction in tafazzin-deficient cells. [PDF]
Liang Z +12 more
europepmc +1 more source
Correction: Stimulating the sir2-spargel axis rescues exercise capacity and mitochondrial respiration in a Drosophila model of Barth syndrome. [PDF]
Damschroder D +5 more
europepmc +1 more source
Adaptive mechanisms in pancreatic islets counteract mitochondrial dysfunction in Barth syndrome. [PDF]
Carlein C +21 more
europepmc +1 more source
SIRT3 regulates cardiolipin biosynthesis in pressure overload-induced cardiac remodeling by PPARγ-mediated mechanism. [PDF]
Liu LX +6 more
europepmc +1 more source
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Tafazzin splice variants and mutations in Barth syndrome
Molecular Genetics and Metabolism, 2014Barth syndrome is caused by mutations in the TAZ (tafazzin) gene on human chromosome Xq28. The human tafazzin gene produces four major mRNA splice variants; two of which have been shown to be functional (TAZ lacking exon 5 and full-length) in complementation studies with yeast and Drosophila.
Susan M, Kirwin +3 more
openaire +2 more sources
Cardiac mitochondrial structure and function in tafazzin-knockdown mice
Mitochondrion, 2018Mutations in the tafazzin gene are the basis of Barth syndrome. The tafazzin protein is responsible for the synthesis of cardiolipin. Doxycycline-induced tafazzin-knockdown mice have been used as a model for Barth syndrome. In the current study, we examined subsarcolemmal and interfibrillar mitochondria from hearts of tafazzin-knockdown mice, focusing ...
Junhwan Kim +4 more
openaire +2 more sources
Cardiolipin remodeling and the function of tafazzin
Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, 2013Cardiolipin, the specific phospholipid of mitochondria, is involved in the biogenesis, the dynamics, and the supramolecular organization of mitochondrial membranes. Cardiolipin acquires a characteristic composition of fatty acids by post-synthetic remodeling, a process that is crucial for cardiolipin homeostasis and function.
openaire +2 more sources
A Reduction in Tafazzin Decreases Mitochondrial Function in C2C12 Myotubes
The FASEB Journal, 2022Mitochondria, which are often regarded as the “powerhouse of the cell”, are labile organelles that regulate cellular metabolism, determine cell fate, and act as important signaling hubs. A phospholipid that is exclusively found within mitochondria that serves numerous roles is cardiolipin (CL).
Brandon J. Richards +2 more
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