Results 61 to 70 of about 2,315 (156)

Stimulating myocardial pyruvate dehydrogenase activity fails to alleviate cardiac abnormalities in a mouse model of human Barth syndrome

Frontiers in Cardiovascular Medicine, 2022
Barth syndrome (BTHS) is a rare genetic disorder due to mutations in the TAFAZZIN gene, leading to impaired maturation of cardiolipin and thereby adversely affecting mitochondrial function and energy metabolism, often resulting in cardiomyopathy.
Amanda A. Greenwell, Amanda A. Greenwell, Amanda A. Greenwell, Seyed Amirhossein Tabatabaei Dakhili, Seyed Amirhossein Tabatabaei Dakhili, Seyed Amirhossein Tabatabaei Dakhili, Keshav Gopal, Keshav Gopal, Keshav Gopal, Christina T. Saed, Christina T. Saed, Christina T. Saed, Jordan S. F. Chan, Jordan S. F. Chan, Jordan S. F. Chan, Nick Kazungu Mugabo, Pavel Zhabyeyev, Pavel Zhabyeyev, Pavel Zhabyeyev, Farah Eaton, Farah Eaton, Farah Eaton, Jennifer Kruger, Gavin Y. Oudit, Gavin Y. Oudit, Gavin Y. Oudit, John R. Ussher, John R. Ussher, John R. Ussher   +28 more
doaj   +1 more source

Mitochondrial Homeostasis in Pancreatic β Cell Function: Mechanisms and Therapeutic Targets for Diabetes

Journal of Diabetes, Volume 18, Issue 5, May 2026.
This review highlights mitochondrial dysfunction as a central driver of pancreatic β cell failure in diabetes, caused by disrupted mitochondrial quality control (MQC), oxidative stress, and impaired organelle communication. Emerging therapies, such as DRAK2 inhibitors and metabolic reprogramming agents, show promise in restoring β cell function by ...
Ruihan Li, Bingqian Zhang, Yuxin Zhang, Yang Yang, Yuting Lu, Jingya Li   +5 more
wiley   +1 more source

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

Microbial Cell, 2018
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 ...
de Tilques, Maxence de Taffin, 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   +10 more
openaire   +6 more sources

Novel drugs approved by the EMA, the FDA and the MHRA in 2025: A year in review

British Journal of Pharmacology, Volume 183, Issue 9, Page 1779-1813, May 2026.
Abstract In the 2025 novel drug mini‐review, one can take a full measure of the ingenuity that underlies current drug design and development, despite the year's smaller harvest (46 novel drugs) compared to 2024 (53) and 2023 (70). 54% of the novel drugs are first‐in‐class (FIC).
Andreas Papapetropoulos, Stavros Topouzis, Steve P. H. Alexander, Miriam M. Cortese‐Krott, Zsuzsanna Helyes, Kirill Martemyanov, Claudio Mauro, Nithyanandan Nagercoil, Reynold A. Panettieri Jr, Hemal H. Patel, Rainer Schulz, Barbara Stefanska, Gary J. Stephens, Nathalie Vergnolle, Xin Wang, Stephen Ward, Péter Ferdinandy   +16 more
wiley   +1 more source

New C-Terminal Conserved Regions of Tafazzin, a Catalyst of Cardiolipin Remodeling [PDF]

Oxidative Medicine and Cellular Longevity, 2019
Cardiolipin interacts with many proteins of the mitochondrial inner membrane and, together with cytochrome C and creatine kinase, activates them. It can be considered as an integrating factor for components of the mitochondrial respiratory chain, which provides for an efficient transfer of electrons and protons.
Gregory A. Shilovsky, Oleg A. Zverkov, Alexandr V. Seliverstov, Vasily V. Ashapkin, Tatyana S. Putyatina, Lev I. Rubanov, Vassily A. Lyubetsky   +6 more
openaire   +2 more sources

Matrifibrocytes Redefine Cardiac Fibrosis: From Terminal Differentiation to Translational Modulation

Journal of Cellular and Molecular Medicine, Volume 30, Issue 10, May 2026.
ABSTRACT Cardiac fibrosis is increasingly recognized as a dynamic program that resolves into a terminal fibroblast fate, the matrifibrocyte, rather than a persistent myofibroblast state. Lineage‐tracing and single‐cell studies reveal that matrifibrocytes arise from activated myofibroblasts during late scar maturation, lose α‐SMA and proliferative ...
Zhentao Zhang, Hua Zhu
wiley   +1 more source

Beneficial effects of SS-31 peptide on cardiac mitochondrial dysfunction in tafazzin knockdown mice

Scientific Reports, 2022
Barth Syndrome (BTHS), a genetic disease associated with early-onset cardioskeletal myopathy, is caused by loss-of-function mutations of the TAFAZZIN gene, which is responsible for remodeling the mitochondrial phospholipid cardiolipin (CL).
Silvia Russo, Domenico De Rasmo, Anna Signorile, Angela Corcelli, Simona Lobasso   +4 more
doaj   +1 more source

Monolysocardiolipin in cultured fibroblasts is a sensitive and specific marker for Barth Syndrome

Journal of Lipid Research, 2006
Barth Syndrome (BTHS) is an X-linked recessive disorder that results in abnormal metabolism of the mitochondrial phospholipid cardiolipin (CL). CLs are decreased and monolysocardiolipins (MLCLs), intermediates in CL metabolism, are increased in a variety
Michiel Adriaan van Werkhoven, David Ross Thorburn, Agi Kyra Gedeon, James Jonathon Pitt   +3 more
doaj   +1 more source

Reduction in mRNA Expression of the Neutrophil Chemoattract Factor CXCL1 in Pseudomonas aeruginosa Treated Barth Syndrome B Lymphoblasts

Biology, 2023
Barth Syndrome (BTHS) is a rare X-linked genetic disease caused by a mutation in the TAFAZZIN gene, which codes for the protein tafazzin involved in cardiolipin remodeling.
Hana M. Zegallai, Kangmin Duan, Grant M. Hatch   +2 more
doaj   +1 more source

BMAL1 Drives Cisplatin Resistance in Non‐Small Cell Lung Cancer Via Lactate‐MRP1 Signaling Pathway

Thoracic Cancer, Volume 17, Issue 8, April 2026.
The drug cisplatin induces BMAL1 expression through AKT signaling in response to cisplatin‐induced oxidative stress. BMAL1 activates HIF‐1α‐mediated metabolic reprogramming and lactate production, which in turn activates the TAZ/c‐Jun/Snail complex to upregulate drug efflux pump MRP1, initiating and sustaining chemoresistance. ABSTRACT Lung cancer, the
Zixin Shi, Ziniu Qin, Chuantao Chen, Xiaolin Yang, Yunhan Hu, Xiaohui Meng, Yuxiang Cao, Xiang Tao, Zhijian Zhang, Tiancheng Xie, Huijun Wei, Zhihao Wu   +11 more
wiley   +1 more source