Results 31 to 40 of about 663 (143)
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
openaire +2 more sources
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
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
doaj +1 more source
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
openaire +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
Archives of Microbiology & Immunology, 2023 Abstract Barth Syndrome is a rare X-linked genetic disorder caused by mutations in the TAFAZZIN gene. We recently demonstrated that tafazzin (Taz) protein deficiency in murine mesenchymal stems (MSCs) reduces immune function of activated wild type (WT) B lymphocytes. Interleukin-10 (
Hana M. Zegallai +2 more
openaire +1 more source
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
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
Monolysocardiolipins accumulate in Barth syndrome but do not lead to enhanced apoptosis
Journal of Lipid Research, 2005 Barth syndrome (BTHS) is an X-linked recessive disorder that is biochemically characterized by low cellular levels of the mitochondrial phospholipid cardiolipin (CL).
Fredoen Valianpour +10 more
doaj +1 more source
Loss of mitochondrial quality control protein Yme1 is deleterious to the viability of yeast lacking tafazzin. [PDF]
, 2014 Role of mitophagy and mitochondrial quality-control in Barth syndrome.The Saccharomyces cerevisiae TAZ1 gene is an orthologue of human TAZ; both encode the protein tafazzin.
Gaspard, Gerard
core +2 more sources