Results 61 to 70 of about 3,950 (204)
Regulation of hepatic cardiolipin metabolism by TNFα: Implication in cancer cachexia [PDF]
, 2015 International audienceCardiolipin (CL) content accumulation leads to an increase in energy wasting in liver mitochondria in a rat model of cancer cachexia in which tumor necrosis factor alpha (TNFα) is highly expressed.
Chevalier, Stephan +12 more
core +3 more sources
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 +10 more
openaire +6 more sources
Blood Advances, 2022 Key Points ER-Hoxb8 conditional immortalization provides a system for the study of TAFAZZIN deficiency in myeloid progenitors and mature neutrophils. Barth syndrome (ie, TAFAZZIN-deficient) murine neutrophils may be more sensitive to apoptosis, possibly ...
Jihee Sohn +13 more
semanticscholar +1 more source
Elevated liver glycogenolysis mediates higher blood glucose during acute exercise in Barth syndrome
PLoS ONE, 2023 Barth syndrome (BTHS) is an X-linked recessive genetic disorder due to mutations in the Tafazzin (TAFAZZIN) gene that lead to cardiac and skeletal muscle mitochondrial dysfunction.
George G. Schweitzer +7 more
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Frontiers in Pediatrics, 2023 BackgroundBarth syndrome is a rare genetic disease characterized by cardiomyopathy, skeletal muscle weakness, neutropenia, growth retardation and organic aciduria.
Nicola Tovaglieri +4 more
doaj +1 more source
Scientific Reports, 2022 Barth syndrome (BTHS) is caused by mutations in the TAZ gene encoding the cardiolipin remodeling enzyme, Tafazzin. The study objective was to quantitatively examine growth characteristics and mitochondrial morphology of transformed lymphoblast cell lines
J. Z. Chan +8 more
semanticscholar +1 more source
The FASEB Journal, 2022 Barth Syndrome (BTHS) is a rare X‐linked genetic disorder caused by mutation in the TAFAZZIN gene. Tafazzin (Taz) deficiency in BTHS patients results in an increased risk of infections.
Hana M. Zegallai +6 more
semanticscholar +1 more source
Modeling the mitochondrial cardiomyopathy of Barth syndrome with iPSC and heart-on-chip technologies [PDF]
, 2015 Studying monogenic mitochondrial cardiomyopathies may yield insights into mitochondrial roles in cardiac development and disease. Here, we combine patient-derived and genetically engineered iPSCs with tissue engineering to elucidate the pathophysiology ...
Agarwal, Ashutosh +27 more
core +1 more source
Neural Domain Adaptation for Biomedical Question Answering
, 2017 Factoid question answering (QA) has recently benefited from the development of deep learning (DL) systems. Neural network models outperform traditional approaches in domains where large datasets exist, such as SQuAD (ca.
Neves, Mariana +2 more
core +1 more source
Mitochondrial dynamism and heart disease: Changing shape and shaping change [PDF]
, 2015 Mitochondria of adult cardiomyocytes appear hypo-dynamic, lacking interconnected reticular networks and the continual fission and fusion observed in many other cell types.
Dorn, Gerald W, II
core +2 more sources