Results 141 to 150 of about 3,950 (204)

Dietary linoleic acid supplementation fails to rescue established cardiomyopathy in Barth syndrome

Journal of Molecular and Cellular Cardiology Plus
Barth syndrome (BTHS) is a mitochondrial lipid disorder caused by mutations in TAFAZZIN (TAZ), required for cardiolipin (CL) remodeling. Cardiomyopathy is a major clinical feature, with no curative therapy.
Siting Zhu, Jing Pang, Anh Nguyen, Helen Huynh, Sharon Lee, Yusu Gu, Frederic M. Vaz, Xi Fang   +7 more
doaj   +1 more source

Stimulating the sir2–spargel axis rescues exercise capacity and mitochondrial respiration in a Drosophila model of Barth syndrome

Disease Models & Mechanisms, 2022
Deena Damschroder, Rubén Zapata-Pérez, Kristin Richardson, Frédéric M. Vaz, Riekelt H. Houtkooper, Robert Wessells   +5 more
doaj   +1 more source

Metabolic cardiomyopathies: untangling clinical heterogeneity with human stem-cell derived models. [PDF]

EMBO Mol Med
Passadouro AS, Balfoort BM, Langeveld M, van Karnebeek CDM, van der Velden J, Houtkooper RH, Mosegaard S.   +6 more
europepmc   +1 more source

Granulopoietic Dysregulation in a Patient-Tailored Mouse Model of Barth Syndrome. [PDF]

Stem Cell Rev Rep
Sierra Potchanant EA, Capitano ML, Edwards DM, Ramdas B, Cooper S, Ropa J, Pay SL, Sheth A, Snider PL, Vernon HJ, Tran NT, Kapur R, Conway SJ.   +12 more
europepmc   +1 more source

Letter to the Editor: CRISPR-based gene editing for cardiac protection in Barth syndrome. [PDF]

Ann Med Surg (Lond)
Abedin ZU, Waggan AI, Khan E, Suleman MU, Tabassum SN.   +4 more
europepmc   +1 more source

De-succinylation-induced accumulation of TRMT10C in the nucleus plays a detrimental role in coronary microembolization via its m1A modification function. [PDF]

Int J Biol Sci
Hu CK, Huang WZ, He L, Chang C, Ren YL, Dai RX, Wu Q, Su Q.   +7 more
europepmc   +1 more source

Transcriptome Analysis of Adipose Tissues from Five Sheep Breeds Reveals Key Genes Involved in Fat Deposition. [PDF]

Genes (Basel)
Yu Y, Liu S, Yang J, Xu S.
europepmc   +1 more source

Upregulation of the AMPK-FOXO1-PDK4 pathway is a primary mechanism of pyruvate dehydrogenase activity reduction and leads to increased glucose uptake in tafazzin-deficient cells

bioRxiv
Zhuqing Liang, Tyler Ralph-Epps, Michael W. Schmidtke, Pablo Lazcano, Simone W. Denis, M. Balážová, Mohamed Chakkour, Sanaa Hazime, Mindong Ren, M. Schlame, R. Houtkooper, M. Greenberg   +11 more
semanticscholar   +1 more source

Integrated multi-omics mapping of mitochondrial dysfunction and substrate preference in Barth syndrome cardiac tissue. [PDF]

EMBO Mol Med
Schomakers BV, Passadouro AS, Trętowicz MM, Simpson PJ, Jaspers YRJ, van Weeghel M, Hu IM, Lamboo CME, Cloutier D, Byrne BJ, van Klinken JB, Janssen PML, Piersma SR, Jimenez CR, Vaz FM, Salomons GS, van der Velden J, Houtkooper RH, Mosegaard S.   +18 more
europepmc   +1 more source

Decreased pyruvate dehydrogenase activity in Tafazzin-deficient cells is caused by dysregulation of pyruvate dehydrogenase phosphatase 1 (PDP1)

Journal of Biological Chemistry
Zhuqing Liang, Tyler Ralph-Epps, Michael W. Schmidtke, Vikalp Kumar, M. Greenberg   +4 more
semanticscholar   +1 more source