Results 31 to 40 of about 3,950 (204)

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

The Function of Tafazzin, a Mitochondrial Phospholipid-Lysophospholipid Acyltransferase.

Journal of Molecular Biology, 2020
Tafazzin is a mitochondrial enzyme that exchanges fatty acids between phospholipids by phospholipid-lysophospholipid transacylation. The reaction alters the molecular species composition and, as a result, the physical properties of lipids. In vivo, the most important substrate of tafazzin is the mitochondria-specific lipid cardiolipin.
M. Schlame, Yang Xu
semanticscholar   +4 more sources

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

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

Author Correction: Upregulation of the AMPK-FOXO1-PDK4 pathway is a primary mechanism of pyruvate dehydrogenase activity reduction in tafazzin-deficient cells. [PDF]

Sci Rep
Liang Z, Ralph-Epps T, Schmidtke MW, Lazcano P, Denis SW, Balážová M, Teixeira da Rosa N, Chakkour M, Hazime S, Ren M, Schlame M, Houtkooper RH, Greenberg ML.   +12 more
europepmc   +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

Cardiac Transplantation Does Not Improve Exercise Tolerance, Muscle Mass, or Substrate Metabolism in Barth Syndrome. [PDF]

JIMD Rep
ABSTRACT Barth syndrome (BTHS) is a rare X‐linked recessive disorder characterized by mutations in the TAFAZZIN gene, leading to mitochondrial dysfunction, cardioskeletal myopathy, neutropenia, exercise intolerance, and growth delay. While cardiac transplantation can improve heart function in BTHS patients, the metabolic effects of this procedure ...
Cade WT, Bohnert KL, Peterson LR, Fuentes LL, Poehlein E, Green CL, Pacak CA, Byrne BJ, Reeds DN, Bashir A, Feingold B, Taylor C.   +11 more
europepmc   +2 more sources