Results 61 to 70 of about 6,556 (165)

Barth Syndrome in Adulthood: A Clinical Case

Revista Española de Cardiología (English Edition), 2013
©2013. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/ This document is the Accepted, version of a Published Work that appeared in final form in Revista Española de Cardiología.
García Molina, Esperanza, Escudero, Fuensanta, Ruiz Espejo, Francisco, Sabater Molina, María, Ballesta Martínez, María Juliana, Guillén Navarro, Encarna   +5 more
openaire   +1 more source

Human trifunctional protein alpha links cardiolipin remodeling to beta-oxidation. [PDF]

PLoS ONE, 2012
Cardiolipin (CL) is a mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes involved in the generation of ATP.
William A Taylor, Edgard M Mejia, Ryan W Mitchell, Patrick C Choy, Genevieve C Sparagna, Grant M Hatch   +5 more
doaj   +1 more source

Bacterial Extracellular Vesicles (BEVs) Derived from Chryseobacterium Inhibit Dengue Virus Infection by Disrupting Its Structural Integrity

Journal of Extracellular Vesicles, Volume 15, Issue 6, June 2026.
This study elucidates a novel antiviral mechanism of bacterial BEVs, which are shown to inactivate viruses by triggering membrane fusion, subsequent lysis and causing structural collapse. This direct virucidal action presents a promising broad‐spectrum strategy against diverse enveloped viruses.
Yaqi Gao, Lijian Zhang, Tianci Zhang, Qiufeng Yao, Ruifang Gao, Yue Wang, Yunpeng Zhao, Tingting Zhou, Jikuai Chen, Xing Zhang, Hao Ren, Yongzhe Zhu, Ping Zhao, Zhongtian Qi, Li Luo, Zhaoling Qin   +15 more
wiley   +1 more source

Redox Regulation and Oxidative Stress in Health and Disease: Mechanisms and Therapeutic Targeting

MedComm – Future Medicine, Volume 5, Issue 2, June 2026.
Reactive species serve crucial roles which are tightly regulated in both physiological as well as disease states. At physiological levels, these species are integral to redox signaling, while uncontrolled redox promotes disease pathology. This review examines the dysregulation of these processes.
Mohammad Hossein Azadi, Azadeh Niknejad, Elaheh Amini   +2 more
wiley   +1 more source

Arginine kinetics are altered in a pilot sample of adolescents and young adults with Barth syndrome

Molecular Genetics and Metabolism Reports, 2020
Barth syndrome (BTHS) is a rare, X-linked cardiomyopathy that is characterized by abnormalities in glucose and lipid metabolism, with less known regarding amino acid metabolism.
W. Todd Cade, Kathryn L. Bohnert, Adam J. Bittel, Shaji J. Chacko, Bruce W. Patterson, Christina A. Pacak, Barry J. Byrne, Hilary J. Vernon, Dominic N. Reeds   +8 more
doaj   +1 more source

Therapeutic Peptide SS‐31 Modulates Membrane Binding and Aggregation of α‐Synuclein and Restores Impaired Mitochondrial Function

Chemical Biology &Drug Design, Volume 107, Issue 6, June 2026.
SS‐31 (Elamipretide) inhibits α‐synuclein aggregation on the membranes and emerges as a promising therapeutic candidate against mitochondrial dysfunction in Parkinson's disease. ABSTRACT Membrane binding and aggregation properties of α‐synuclein are closely associated with Parkinson's disease and a class of related syndromes named as synucleinopathy ...
Ewelina Stefaniak, Beiyuan Cui, Xucheng Yan, Kexin Sun, Xiangyu Teng, Liming Ying   +5 more
wiley   +1 more source

Mouse Tafazzin Is Required for Male Germ Cell Meiosis and Spermatogenesis.

PLoS ONE, 2015
Barth syndrome is an X-linked mitochondrial disease, symptoms of which include neutropenia and cardiac myopathy. These symptoms are the most significant clinical consequences of a disease, which is increasingly recognised to have a variable presentation.
Laurence C Cadalbert, Farah Naz Ghaffar, David Stevenson, Sheila Bryson, Frédéric M Vaz, Eyal Gottlieb, Douglas Strathdee   +6 more
doaj   +1 more source

Dynamic simulation of cardiolipin remodeling: greasing the wheels for an interpretative approach to lipidomics[S]

Journal of Lipid Research, 2010
Cardiolipin is a class of mitochondrial specific phospholipid, which is intricately involved in mitochondrial functionality. Differences in cardiolipin species exist in a variety of tissues and diseases.
Michael A. Kiebish, Rob Bell, Kui Yang, Toan Phan, Zhongdan Zhao, William Ames, Thomas N. Seyfried, Richard W. Gross, Jeffrey H. Chuang, Xianlin Han   +9 more
doaj   +1 more source

Generation of urine-derived iPS cell line via a non-integrative method from a Barth syndrome patient with TAZ gene mutation

Stem Cell Research, 2020
Human urine cells from a 6-year-old male X-linked Barth syndrome patient harboring a TAZ frameshift (c.517delG, Xq28) were reprogrammed into the induced pluripotent stem cell (iPSC) line WMUi002-A using non-integration CytoTune®-iPS 2.0 Sendai Virus ...
Xiaoling Guo, Long Wang, Kaixin Chen, Shiyang Song, Xingang Wang, Xiaohong Gu, Chao Niu, Maoping Chu   +7 more
doaj   +1 more source

From Pharmacodynamic Biomarker to Evaluating Treatment Response: Biomarkers in Primary Mitochondrial Diseases

Clinical and Translational Science, Volume 19, Issue 6, June 2026.
ABSTRACT Primary mitochondrial diseases (PMDs) result from genetic variants in nuclear DNA and mitochondrial DNA which commonly lead to aberrant oxidative phosphorylation. The clinical complexity, often attributed to the underlying genetics, includes several distinct syndromes (e.g., Barth syndrome; Pearson syndrome; Mitochondrial encephalomyopathy ...
Sydney Stern, Karryn Crisamore, John Patton, Robert Schuck, Michael Pacanowski   +4 more
wiley   +1 more source