Results 81 to 90 of about 2,732 (148)

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

, 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).
Godard, François, de Tilques, Maxence de Taffin, Lasserre, Jean-Paul, di Rago, Jean-Paul, Marine Bouhier, Sardin, Elodie, Elodie Sardin, Eric Testet, Bouhier, Marine, de Taffin de Tilques, Maxence, Testet, Eric, Tribouillard-Tanvier, Déborah, Déborah Tribouillard-Tanvier, Jean-Paul Lasserre, Roza Kucharczyk, François Godard, Marina Le Guedard, Le Guedard, Marina, Kucharczyk, Roza, Petit, Patrice X., Patrice X. Petit, Jean-Paul di Rago, Maxence de Taffin de Tilques   +22 more
core   +1 more source

Cardiac‐specific succinate dehydrogenase deficiency in Barth syndrome

EMBO Molecular Medicine, 2015
Barth syndrome (BTHS) is a cardiomyopathy caused by the loss of tafazzin, a mitochondrial acyltransferase involved in the maturation of the glycerophospholipid cardiolipin.
Jan Dudek, I‐Fen Cheng, Arpita Chowdhury, Katharina Wozny, Martina Balleininger, Robert Reinhold, Silke Grunau, Sylvie Callegari, Karl Toischer, Ronald JA Wanders, Gerd Hasenfuß, Britta Brügger, Kaomei Guan, Peter Rehling   +13 more
doaj   +1 more source

Cardiolipin biosynthesis and remodeling enzymes are altered during development of heart failure

Journal of Lipid Research, 2009
Cardiolipin (CL) is responsible for modulation of activities of various enzymes involved in oxidative phosphorylation. Although energy production decreases in heart failure (HF), regulation of cardiolipin during HF development is unknown.
Harjot K. Saini-Chohan, Michael G. Holmes, Adam J. Chicco, William A. Taylor, Russell L. Moore, Sylvia A. McCune, Diane L. Hickson-Bick, Grant M. Hatch, Genevieve C. Sparagna   +8 more
doaj   +1 more source

DataSheet2_Re-Expression of Tafazzin Isoforms in TAZ-Deficient C6 Glioma Cells Restores Cardiolipin Composition but Not Proliferation Rate and Alterations in Gene Expression.pdf

, 2022
Tafazzin—an acyltransferase—is involved in cardiolipin (CL) remodeling. CL is associated with mitochondrial function, structure and more recently with cell proliferation. Various tafazzin isoforms exist in humans.
Michael Linnebacher (180341), Daniela Peter (13146513), Stefan Simm (277838), Michael Lalk (117041), Uwe Lendeckel (4405486), Gayatri Jagirdar (13146510), Mathias Krohn (443089), Christian Scharf (104359), Matthias Elsner (3507983), Karen Methling (510762), Katrin Borucki (229301), Carmen Wolke (13146516)   +11 more
core   +1 more source

Identification of a Novel Gene Mutation in a Family With X-Linked Dilated Cardiomyopathy Barth Syndrome

Journal of Inborn Errors of Metabolism and Screening, 2015
Mutations in the tafazzin ( TAZ ) gene on chromosome Xq28 are responsible for the Barth syndrome (BTHS) phenotype resulting in a loss of function in the protein tafazzin involved in the transacylation of cardiolipin, an essential mitochondrial ...
Minal Borkar PhD, Sunita Bijarnia-Mahay DCH, DNB, MD, Sudha Kohli PhD, Monica Juneja MD, Yogesh Srivastava M.Sc, Renu Saxena PhD, Ishwar C. Verma FRCP   +6 more
doaj   +1 more source

Cardiac and Skeletal Muscle Defects in a Mouse Model of Human Barth Syndrome

, 2011
Barth syndrome is an X-linked genetic disorder caused by mutations in the tafazzin (taz) gene and characterized by dilated cardiomyopathy, exercise intolerance, chronic fatigue, delayed growth, and neutropenia.
Moore, Vicky, Wansapura, Janaka, Kulik, Willem, James, Jeanne, Vaz, Frederic, Acehan, Devrim, Toth, Matthew J., Houtkooper, Riekelt H., Khuchua, Zaza, Tokunaga, Chonan, Strauss, Arnold   +10 more
core   +2 more sources

Tafazzin knockdown and Dox-sensitivity in H9c2 cardiomyocytes.

, 2016
(A) knockdown of tafazzin was confirmed in H9c2 cells after transfection with 20 nM tafazzin siRNA for 48 h by western blot analysis. (B) Representative western blot showing the level of cleaved caspase-3.
Baikuntha Aryal (441940), V. Ashutosh Rao (441950)   +1 more
core   +1 more source

Role of Hippo Pathway Effector Tafazzin Protein in Maintaining Stemness of Umbilical Cord-Derived Mesenchymal Stem Cells (UC-MSC) [Elektronisk resurs]

, 2018
Tafazzin (TAZ) protein has been upregulated in various types of human cancers, although the basis for elevation is uncertain, it has been made definite that the effect of mutation in the hippo pathway, particularly when it is switched off, considerably ...
Gopinath, Madhumala,, Marotta, Francesco,, Sun, Xiao-Feng,, Subramaniam, Vimala Devi,, Narasimhan, Srinivasan,, Priya K, Swarna,, Murugesan, Ramachandran,, Banerjee, Antara,, Sriramulu, Sushmitha,, Di Liddo, Rosa,, Jothimani, Ganesan,, Pathak, Surajit,   +11 more
core  

Barth syndrome: mechanisms and management

The Application of Clinical Genetics, 2019
Josef FinstererKrankenanstalt Rudolfstiftung, Messerli Institute, Vienna, AustriaObjectives: Barth syndrome is an ultra-rare, infantile-onset, X-linked recessive mitochondrial disorder, primarily affecting males, due to variants in TAZ encoding for the ...
Finsterer J
doaj  

Stimulating the sir2–spargel axis rescues exercise capacity and mitochondrial respiration in a Drosophila model of Barth syndrome

Disease Models & Mechanisms, 2022
Deena Damschroder, Rubén Zapata-Pérez, Kristin Richardson, Frédéric M. Vaz, Riekelt H. Houtkooper, Robert Wessells   +5 more
doaj   +1 more source