Petit 2018 MiP2018: Difference between revisions
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Decreasing cytosolic translation is beneficial to yeast and human Tafazzin-deficient cells with dirupted mitochondrial homeostasis (Barth syndrome cell model). |
Link: MiP2018
De Taffin de Tilques M, Lasserre JP, Godard F, Sardin E, Bouhier M, Le Guedard M, Kucharczyk R, Testet E, Di Rago JP, Tribouillard-Tanvier D, Petit PX (2018)
Event: MiP2018
Cardiolipin (CL) optimizes diverse mitochondrial processes, includ-ing oxidative phosphorylation (OXPHOS). To function properly, CL needs to be unsaturated, which requires the acyltransferase Tafazzin (TAZ) [1]. Loss-of-function mutations in the TAZ gene are responsible for the Barth syndrome (BTHS), a rare X-linked cardiomyopathy, presumably because of a diminished OXPHOS capacity [2]. Herein we show that a partial inhibition of cytosolic protein synthesis, either chemically with the use of cycloheximide or by specific genetic mutations, fully restores biogenesis and the activity of the oxidative phosphorylation system in a yeast BTHS model (taz1Ξ). Interestingly, the defaults in CL were not suppressed, indicating that they are not primarily responsible for the OXPHOS deficiency in taz1Ξ yeast. Low concentrations of cycloheximide in the picomolar range were beneficial to TAZ-deficient HeLa cells, as evidenced by the recovery of a good proliferative capacity. These findings reveal that a diminished capacity of CL remodeling deficient cells to preserve protein homeostasis is likely an important factor contributing to the pathogenesis of BTHS. This in turn, identifies cytosolic translation as a potential therapeutic target for the treatment of this disease [3]
β’ Bioblast editor: Plangger M, Kandolf G
Labels: MiParea: nDNA;cell genetics Pathology: Myopathy
Organism: Human, Saccharomyces cerevisiae Tissue;cell: HeLa
Affiliations
De Taffin de Tilques M(2), Lasserre JP(2), Godard F, Sardin E, Bouhier M, Le Guedard M, Kucharczyk R, Testet E, Di Rago JP(2), Tribouillard-Tanvier D(2), Petit PX(1)
- CNRS FR3636, Institut de Neurosciences, Univ Paris-Descartes, France
- Inst de Biochimie et gΓ©nΓ©tique cellulaire, CNRS UMR 5095, Univ de Bordeaux, France.
References
- Saric A, β¦ Petit PX (2016) Front Genet 20;6:359.
- Gonzalvez F, β¦ Petit PX (2013) Biochim Biophys Acta; 1832 (8): 1194-206.
- De Taffin de Tilques M, β¦ Tribouillard-Tanvier D (2018) Microb Cell 18;5(5):220-32.