Object's details: Decreasing cytosolic translation is beneficial to yeast and human Tafazzin-deficient cells

Provider:Repozytorium Instytutu Biochemii i Biofizyki Polskiej Akademii Nauk

Description

Title:
Decreasing cytosolic translation is beneficial to yeast and human Tafazzin-deficient cells
Creator:
de Taffin de Tilques, Maxence;
Lasserre, Jean-Paul;
Godard, François;
Sardin, Elodie;
Bouhier, Marine;
Le Guedard, Marina;
Kucharczyk, Roza;
Petit, Patrice X.;
Testet, Eric;
di Rago, Jean-Paul;
Tribouillard-Tanvier, Déborah
Description:
Cardiolipin (CL) optimizes diverse mitochondrial processes, including oxidative phosphorylation (OXPHOS). To function properly, CL needs to be unsaturated, which requires the acyltransferase Tafazzin (TAZ). 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. 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 (taz1Δ). Interestingly, the defaults in CL were not suppressed, indicating that they are not primarily responsible for the OXPHOS deficiency in taz1Δ 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.
Object availability:
free access
Date:
2018.02
Type:
Article;
PeerReviewed
Language:
English;
eng
Format:
application/pdf
Relation: http://eprints.ibb.waw.pl/1553 ;http://doi.org/10.15698/mic2018.05.629 ;doi:10.15698/mic2018.05.629
Subject:
Q Science (General);
RB Pathology
Identifier: http://eprints.ibb.waw.pl/1553/1/Taffin_micrCell2018.pdf ;
de Taffin de Tilques, Maxence and Lasserre, Jean-Paul and Godard, François and Sardin, Elodie and Bouhier, Marine and Le Guedard, Marina and Kucharczyk, Roza and Petit, Patrice X. and Testet, Eric and di Rago, Jean-Paul and Tribouillard-Tanvier, Déborah (2018) Decreasing cytosolic translation is beneficial to yeast and human Tafazzin-deficient cells. Microbial Cell, 5 (5). pp. 220-232. ISSN 23112638
Data provider:
Repozytorium Instytutu Biochemii i Biofizyki Polskiej Akademii Nauk

Similar objects

Decreasing cytosolic translation is beneficial to yeast and human Tafazzin-deficient cells

Date:2018.02

Type:other

Proteomic Profile of Saliva in Parkinson’s Disease Patients: A Proof of Concept Study

Date:2021.05.18

Type:other

Molecular Basis of the Pathogenic Mechanism Induced by the m.9191T>C Mutation in Mitochondrial ATP6 Gene

Date:2020

Type:other

Ni(II) ions cleave and inactivate human alpha-1 antitrypsin hydrolytically, implicating nickel exposure as a contributing factor in pathologies related to antitrypsin deficiency

Creator:Wezynfeld, Nina Ewa | Bonna, Arkadiusz | Bal, Wojciech | Frączyk, Tomasz

Date:2015

Type:other

Unusual Zn(II) Affinities of Zinc Fingers of Poly(ADP-ribose) Polymerase 1 (PARP-1) Nuclear Protein

Date:2015.12.30

Type:other

ATP Synthase Diseases of Mitochondrial Genetic Origin

Date:2018

Type:other

Flavonoids as Potential Drugs for VPS13-Dependent Rare Neurodegenerative Diseases

Date:2020

Type:other

Defining the impact on yeast ATP synthase of two pathogenic human mitochondrial DNA mutations, T9185C and T9191C

Creator:Kabala, Anna Magdalena | Lasserre, Jean-Paul | Ackerman, Sharon H. | di Rago, Jean-Paul | Kucharczyk, Roza

Date:2014

Type:other

Federacja Bibliotek Cyfrowych

Object's details: Decreasing cytosolic translation is beneficial to yeast and human Tafazzin-deficient cells

Description

Similar objects