Stimulating the sir2-spargel axis rescues exercise capacity and mitochondrial respiration in a Drosophila model of Barth syndrome.
Stimulating the sir2-spargel axis rescues exercise capacity and mitochondrial respiration in a Drosophila model of Barth syndrome.
复制标题
DOI：
10.1242/dmm.049279
复制
发表时间：
2022-10-01
影响因子：
4.3
通讯作者：
中科院分区：
文献类型：
--
作者：
Cardiolipin (CL) is a phospholipid required for proper mitochondrial function. Tafazzin remodels CL to create highly unsaturated fatty acid chains. However, when TAFAZZIN is mutated, CL remodeling is impeded, leading to mitochondrial dysfunction and the disease Barth syndrome. Patients with Barth syndrome often have severe exercise intolerance, which negatively impacts their overall quality of life. Boosting NAD+ levels can improve symptoms of other mitochondrial diseases, but its effect in the context of Barth syndrome has not been examined. We demonstrate, for the first time, that nicotinamide riboside can rescue exercise tolerance and mitochondrial respiration in a Drosophila Tafazzin mutant and that the beneficial effects are dependent on sir2 and spargel. Overexpressing spargel increased the total abundance of CL in mutants. In addition, muscles and neurons were identified as key targets for future therapies because sir2 or spargel overexpression in either of these tissues is sufficient to restore the exercise capacity of Drosophila Tafazzin mutants. Summary: Nicotinamide riboside rescues the exercise capacity and mitochondrial function of a Drosophila model of Barth syndrome in a Sir2/Spargel-dependent manner.
登录
查看更多内容
血浆NAD（+）代谢组在“正常”衰老中失调。
影响因子：
2.6
作者：
Clement J;Wong M;Poljak A;Sachdev P;Braidy N
通讯作者：
Braidy N
影响因子：
8.1
作者：
Amjad S;Nisar S;Bhat AA;Shah AR;Frenneaux MP;Fakhro K;Haris M;Reddy R;Patay Z;Baur J;Bagga P
通讯作者：
Bagga P
DOI：
10.1007/8904_2016_553
发表时间：
2017-01-01
期刊：
JIMD REPORTS, VOL 32
影响因子：
--
作者：
Cade, W. Todd;Reeds, Dominic N.;Taylor, Carolyn L.
通讯作者：
Taylor, Carolyn L.
影响因子：
4.4
作者：
BARTH, PG;SCHOLTE, HR;SOBOTKAPLOJHAR, MA
通讯作者：
SOBOTKAPLOJHAR, MA
DOI：
10.1016/j.tem.2021.01.006
发表时间：
2021-04
期刊：
Trends in endocrinology and metabolism: TEM
影响因子：
--
作者：
Falabella M;Vernon HJ;Hanna MG;Claypool SM;Pitceathly RDS
通讯作者：
Pitceathly RDS