Cargando…

Drosophila tafazzin mutants have impaired exercise capacity

Cardiolipin (CL) is a mitochondrial phospholipid that helps maintain normal structure of the inner mitochondrial membrane and stabilize the protein complexes of the electron transport chain to promote efficient ATP synthesis. Tafazzin, an acyl‐transferase, is required for synthesis of the mature for...

Descripción completa

Detalles Bibliográficos
Autores principales: Damschroder, Deena, Reynolds, Christian, Wessells, Robert
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5800294/
https://www.ncbi.nlm.nih.gov/pubmed/29405656
http://dx.doi.org/10.14814/phy2.13604
_version_ 1783298186801053696
author Damschroder, Deena
Reynolds, Christian
Wessells, Robert
author_facet Damschroder, Deena
Reynolds, Christian
Wessells, Robert
author_sort Damschroder, Deena
collection PubMed
description Cardiolipin (CL) is a mitochondrial phospholipid that helps maintain normal structure of the inner mitochondrial membrane and stabilize the protein complexes of the electron transport chain to promote efficient ATP synthesis. Tafazzin, an acyl‐transferase, is required for synthesis of the mature form of CL. Mutations in the tafazzin (TAZ) gene are associated with a human disorder known as Barth syndrome. Symptoms of Barth syndrome often include muscle weakness and exercise intolerance. Previous work demonstrates that Drosophila Taz mutants exhibit motor weakness, as measured by reduced flying and climbing abilities. However, Drosophila TAZ mutants’ baseline endurance or response to endurance exercise training has not been assessed. Here, we find that TAZ mutants have reduced endurance and do not improve following a stereotypical exercise training paradigm, indicating that loss of TAZ function leads to exercise intolerance in Drosophila. Although cardiac phenotypes are observed in human Barth syndrome patients, TAZ mutants had normal resistance to cardiac pacing. In the future, endurance may be a useful screening tool to identify additional genetic modifiers of tafazzin.
format Online
Article
Text
id pubmed-5800294
institution National Center for Biotechnology Information
language English
publishDate 2018
publisher John Wiley and Sons Inc.
record_format MEDLINE/PubMed
spelling pubmed-58002942018-03-15 Drosophila tafazzin mutants have impaired exercise capacity Damschroder, Deena Reynolds, Christian Wessells, Robert Physiol Rep Original Research Cardiolipin (CL) is a mitochondrial phospholipid that helps maintain normal structure of the inner mitochondrial membrane and stabilize the protein complexes of the electron transport chain to promote efficient ATP synthesis. Tafazzin, an acyl‐transferase, is required for synthesis of the mature form of CL. Mutations in the tafazzin (TAZ) gene are associated with a human disorder known as Barth syndrome. Symptoms of Barth syndrome often include muscle weakness and exercise intolerance. Previous work demonstrates that Drosophila Taz mutants exhibit motor weakness, as measured by reduced flying and climbing abilities. However, Drosophila TAZ mutants’ baseline endurance or response to endurance exercise training has not been assessed. Here, we find that TAZ mutants have reduced endurance and do not improve following a stereotypical exercise training paradigm, indicating that loss of TAZ function leads to exercise intolerance in Drosophila. Although cardiac phenotypes are observed in human Barth syndrome patients, TAZ mutants had normal resistance to cardiac pacing. In the future, endurance may be a useful screening tool to identify additional genetic modifiers of tafazzin. John Wiley and Sons Inc. 2018-02-06 /pmc/articles/PMC5800294/ /pubmed/29405656 http://dx.doi.org/10.14814/phy2.13604 Text en © 2018 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society. This is an open access article under the terms of the Creative Commons Attribution (http://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Research
Damschroder, Deena
Reynolds, Christian
Wessells, Robert
Drosophila tafazzin mutants have impaired exercise capacity
title Drosophila tafazzin mutants have impaired exercise capacity
title_full Drosophila tafazzin mutants have impaired exercise capacity
title_fullStr Drosophila tafazzin mutants have impaired exercise capacity
title_full_unstemmed Drosophila tafazzin mutants have impaired exercise capacity
title_short Drosophila tafazzin mutants have impaired exercise capacity
title_sort drosophila tafazzin mutants have impaired exercise capacity
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5800294/
https://www.ncbi.nlm.nih.gov/pubmed/29405656
http://dx.doi.org/10.14814/phy2.13604
work_keys_str_mv AT damschroderdeena drosophilatafazzinmutantshaveimpairedexercisecapacity
AT reynoldschristian drosophilatafazzinmutantshaveimpairedexercisecapacity
AT wessellsrobert drosophilatafazzinmutantshaveimpairedexercisecapacity