Results 21 to 30 of about 3,514 (179)

Tafazzin senses curvature [PDF]

Nature Chemical Biology, 2012
The phospholipid-lysophospholipid transacylase tafazzin is responsible for enrichment of the cardiolipin fraction of mitochondria with tetralinoleoyl-cardiolipin. The specificity for linoleoyl hydrocarbon chains is now explained by the specific action of tafazzin on negatively curved lipid monolayers.
Michael, Schlame, Devrim, Acehan, Bob, Berno, Yang, Xu, Salvatore, Valvo, Mindong, Ren, David L, Stokes, Richard M, Epand   +7 more
  +8 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, Robert W E Crozier, Katie D Hunter, Steven M Claypool, Val A Fajardo, Paul J LeBlanc, Adam J MacNeil   +6 more
openaire   +2 more sources

The Enzymatic Function of Tafazzin [PDF]

Journal of Biological Chemistry, 2006
Tafazzin is a putative enzyme that is involved in cardiolipin metabolism, it may carry mutations responsible for Barth syndrome. To identify the biochemical reaction catalyzed by tafazzin, we expressed the full-length isoform of Drosophila melanogaster tafazzin in a baculovirus-Sf9 insect cell system.
Yang, Xu, Ashim, Malhotra, Mindong, Ren, Michael, Schlame   +3 more
openaire   +2 more sources

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, Reynolds, Christian, Wessells, Robert   +2 more
openaire   +2 more sources

The Influence of Supplemental Dietary Linoleic Acid on Skeletal Muscle Contractile Function in a Rodent Model of Barth Syndrome

Frontiers in Physiology, 2021
Barth syndrome is a rare and incurable X-linked (male-specific) genetic disease that affects the protein tafazzin (Taz). Taz is an important enzyme responsible for synthesizing biologically relevant cardiolipin (for heart and skeletal muscle, cardiolipin
Mario Elkes, Martin Andonovski, Daislyn Vidal, Madison Farago, Ryan Modafferi, Steven M. Claypool, Paul J. LeBlanc   +6 more
doaj   +1 more source

A murine model of Barth syndrome recapitulates human cardiac and skeletal muscle phenotypes [PDF]

Disease Models & Mechanisms
Erika Yazawa, Erin M. Keating, Suya Wang, Mason E. Sweat, Qing Ma, Yang Xu, Michael Schlame, William T. Pu   +7 more
doaj   +2 more sources

Tafazzin Mutation Affecting Cardiolipin Leads to Increased Mitochondrial Superoxide Anions and Mitophagy Inhibition in Barth Syndrome

Cells, 2020
Tafazzin is a phospholipid transacylase that catalyzes the remodeling of cardiolipin, a mitochondrial phospholipid required for oxidative phosphorylation.
Patrice X. Petit, Hector Ardilla-Osorio, Lucile Penalvia, Nathan E. Rainey   +3 more
doaj   +1 more source

Expanding the phenotypic and genetic landscape of congenital neutropenia through whole-exome and genome sequencing. [PDF]

Hemasphere
Abstract Congenital neutropenia (CN) comprises a heterogeneous group of rare genetic disorders. While some CN cases present only with neutropenia, others present with additional extra‐hematological manifestations. The most common cause of CN is variants in ELANE; however, approximately 30 other genes have been implicated.
Marti S, Pellet P, Beaupain B, Durix L, Buratti J, Réguerre Y, Aladjidi N, Azarnoush S, Clauin S, Chahla WA, Blaison G, Bertand J, Bodet D, Brethon B, Chane-Teng J, Delafoy M, Dupraz C, Gandemer V, Denizeau P, Goldenberg A, Hirsch P, l'Haridon A, Marie-Cardine A, Vera G, Nelken B, Nizery L, Nolla M, Pasquet M, Rosain J, Terriou L, Plo I, Donadieu J, Bellanné-Chantelot C.   +32 more
europepmc   +2 more sources

Prenatal case report of Barth syndrome caused by novel TAFAZZIN mutation: Clinical characteristics of fetal dilated cardiomyopathy with ascites

Frontiers in Pediatrics, 2022
Barth syndrome (BTHS) is a rare X-linked recessive genetic disease, which appears in infancy with myocardial and skeletal muscle diseases, neutropenia, growth retardation, and other clinical features.
Xuliang Zhao, Xu Li, Weiwei Sun, Jian-an Jia, Min Yu, Ruixia Tian   +5 more
doaj   +1 more source

The Basis for Acyl Specificity in the Tafazzin Reaction [PDF]

Journal of Biological Chemistry, 2017
Tafazzin is a mitochondrial enzyme that transfers fatty acids from phospholipids to lysophospholipids. Mutations in tafazzin cause abnormal molecular species of cardiolipin and the clinical phenotype of Barth syndrome. However, the mechanism by which tafazzin creates acyl specificity has been controversial.
Michael, Schlame, Yang, Xu, Mindong, Ren
openaire   +2 more sources