Results 41 to 50 of about 2,620 (178)
Studying Lipid-Related Pathophysiology Using the Yeast Model
Frontiers in Physiology, 2021 Saccharomyces cerevisiae, commonly known as baker’s yeast, is one of the most comprehensively studied model organisms in science. Yeast has been used to study a wide variety of human diseases, and the yeast model system has proved to be an especially ...
Tyler Ralph-Epps +5 more
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Drosophila tafazzinmutants have impaired exercise capacity [PDF]
Physiological Reports, 2018 Cardiolipin (CL) is a mitochondrial phospholipid that helps maintain normal structure of the inner mitochondrial membrane and stabilize the protein complexes of the electron transport chain to promote efficient ATP synthesis. Tafazzin, an acyl-transferase, is required for synthesis of the mature form of CL.
Damschroder, Deena +2 more
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Effects of N‐oleoylethanolamide on Lymphoblasts Deficient in Tafazzin
The FASEB Journal, 2021 Barth Syndrome (BTHS) is a rare X‐linked genetic disorder caused by mutations in the TAZ gene that encodes for the cardiolipin remodelling enzyme, Tafazzin. This syndrome is characterized by cardiac and skeletal myopathies, as well as immunological deficits that cause significant morbidity ...
John Chan +6 more
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Phosphatidylglycerol Supplementation Alters Mitochondrial Morphology and Cardiolipin Composition
Membranes, 2022 The pathogenic variant of the TAZ gene is directly associated with Barth syndrome. Because tafazzin in the mitochondria is responsible for cardiolipin (CL) remodeling, all molecules related to the metabolism of CL can affect or be affected by TAZ ...
I Chu +6 more
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Exercise tolerance with Tafazzin knockdown.
, 2023 A, Expression of Tafazzin in vastus, heart, or liver of WT and TG mice after undergoing exercise studies shown in this figure. B, Treadmill exercise time to exhaustion for WT and TG mice.
Christina A. Pacak (16914168) +7 more
core +1 more source
Mouse Tafazzin Is Required for Male Germ Cell Meiosis and Spermatogenesis [PDF]
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.
Cadalbert, L.C. +6 more
openaire +5 more sources
Tafazzin Modulates Allergen-Induced Mast Cell Inflammatory Mediator Secretion [PDF]
ImmunoHorizons, 2021 Abstract Allergic inflammatory diseases are a steadily growing health concern. Mast cells, a driving force behind allergic pathologies, modulate metabolic pathways to carry out various functions following IgE-FcεRI–mediated activation. Tafazzin (TAZ) is a cardiolipin transacylase that functions to remodel, and thereby mature, cardiolipin,
Aindriu R R Maguire +6 more
openaire +2 more sources
Defective Mitochondrial Cardiolipin Remodeling Dampens HIF-1α Expression in Hypoxia
Cell Reports, 2018 Summary: Mitochondria fulfill vital metabolic functions and act as crucial cellular signaling hubs, integrating their metabolic status into the cellular context.
Arpita Chowdhury +17 more
doaj +1 more source
Molecular Therapy: Methods & Clinical Development, 2019 Barth syndrome (BTHS) is a rare mitochondrial disease that causes severe cardiomyopathy and has no disease-modifying therapy. It is caused by recessive mutations in the gene tafazzin (TAZ), which encodes tafazzin—an acyltransferase that remodels the ...
Silveli Suzuki-Hatano +6 more
doaj +1 more source
Frontiers in Molecular Biosciences, 2022 Barth syndrome (BTHS, OMIM 302060) is a genetic disorder caused by variants of the TAFAZZIN gene (G 4.5, OMIM 300394). This debilitating disorder is characterized by cardio- and skeletal myopathy, exercise intolerance, and neutropenia.
Zhuqing Liang +2 more
doaj +1 more source