Results 41 to 50 of about 4,774 (217)

Defining functional classes of Barth syndrome mutation in humans [PDF]

, 2016
The X-linked disease Barth syndrome (BTHS) is caused by mutations in TAZ; TAZ is the main determinant of the final acyl chain composition of the mitochondrial-specific phospholipid, cardiolipin.
Claypool, Steven M., Galbraith, Laura, Gottlieb, Eyal, Herndon, Jenny, Koehler, Carla M., Lu, Ya-Lin, Lu, Ya-Wen, Luyf, Angela, McCaffery, J.Michael, Pras-Raves, Mia, Van Kampen, Antoine, Vaz, Frederic M., Vervaart, Martin   +12 more
core   +10 more sources

Osmotic shock stimulates de novo synthesis of two cardiolipins in an extreme halophilic archaeon

Journal of Lipid Research, 2004
The present report illustrates the response to osmotic stress of an extreme halophilic archaeon, Halorubrum sp., isolated from the saltern ponds of Margherita di Savoia in southern Italy.
Patrizia Lopalco, Simona Lobasso, Francesco Babudri, Angela Corcelli   +3 more
doaj   +1 more source

Oxidative Stress Linked Organ Lipid Hydroperoxidation and Dysregulation in Mouse Model of Nonalcoholic Steatohepatitis: Revealed by Lipidomic Profiling of Liver and Kidney

Antioxidants, 2021
Nonalcoholic steatohepatitis (NASH) is a prevalent disease related to lipid metabolism disorder and oxidative stress. Lipid hydroperoxidation is known to be a critical driving force of various disorders and diseases.
Yue Wu, Zhen Chen, Hirotoshi Fuda, Takayuki Tsukui, Xunzhi Wu, Nianqiu Shen, Natsuki Saito, Hitoshi Chiba, Shu-Ping Hui   +8 more
doaj   +1 more source

Diminished exercise capacity and mitochondrial bc1 complex deficiency in tafazzin-knockdown mice.

Frontiers in Physiology, 2013
The phospholipid, cardiolipin, is essential for maintaining mitochondrial structure and optimal function. Cardiolipin-deficiency in humans, Barth syndrome, is characterized by exercise intolerance, dilated cardiomyopathy, neutropenia and 3-methyl ...
Corey ePowers, Yan eHuang, Arnold W Strauss, Zaza eKhuchua   +3 more
doaj   +1 more source

Recent advances in expanding the coverage of the lipidome [PDF]

, 2017
The lipidome comprises a large array of molecules with diverse physicochemical properties. Lipids are structural components of cells, act as a source of energy, and function as signaling mediators.
Kamphorst, Jurre J., Tumanov, Sergey
core   +1 more source

Structure of a mitochondrial ATP synthase with bound native cardiolipin

eLife, 2019
The mitochondrial ATP synthase fuels eukaryotic cells with chemical energy. Here we report the cryo-EM structure of a divergent ATP synthase dimer from mitochondria of Euglena gracilis, a member of the phylum Euglenozoa that also includes human parasites.
Alexander Mühleip, Sarah E McComas, Alexey Amunts   +2 more
doaj   +1 more source

Metabolomic Profiling in the Characterization of Degenerative Bone and Joint Diseases

Metabolites, 2020
Osteoarthritis and inflammatory arthropathies are a cause of significant morbidity globally. New research elucidating the metabolic derangements associated with a variety of bone and joint disorders implicates various local and systemic metabolites ...
Katherine R. Swank, Jamie E. Furness, Erin A. Baker, Corinn K. Gehrke, Stephen P. Biebelhausen, Kevin C. Baker   +5 more
doaj   +1 more source

Mouse mitochondrial lipid composition is defined by age in brain and muscle [PDF]

, 2017
Functionality of the lipid rich mitochondrial organelle declines with increased age. Recent advances in lipidomic technologies allowed us to perform a global characterisation of lipid composition in two different tissue types and age ranges.
Al-Gubory, Arnould, Becker, Brügger, Chakrabarti, Chakrabarti, Cherkaoui-Malki, Coleman, Corcelli, Daum, Eckert, Goto, Gottlieb, Ha, Han, Harman, Hulbert, Klein-Seetharaman, Koehler, Koltai, Kristal, Lavialle, Liu, Liu, Madeo, McQuibban, Moreau, Nakaya, Nicolaou, Nunnari, Osiewacz, Patel, Pufa, Rea, Rudich, Schägger, Sonninen, Tasseva, Vaz, Verkhratsky, Vidal-Puig, Wenk   +41 more
core   +1 more source

LL‐37 Driven Phase Transition and Stacking in Oligolamellar Gram‐Negative Bacterial Membrane Models

Advanced Functional Materials, EarlyView.
This work establishes oligolamellar bacterial membrane models to investigate how LL‐37 disrupts the complex dual‐bilayer architecture of Gram‐negative bacteria. Combining SAXS, cryo‐TEM, electrophoretic mobility measurements, and coarse‐grained simulations, it reveals cardiolipin‐driven phase transitions leading to bicelle‐like structures and membrane ...
Bettina Tran, Diane C.A. Lima, Linda Sandblad, Madeleine Ramstedt, Thereza A. Soares, Stefan Salentinig   +5 more
wiley   +1 more source

Nature‐Inspired Surface Modification Strategy Reverses the Autophagic Flux Impairment of Mitochondrial Transplantation for Attenuating Ischemic Strokes

Advanced Science, EarlyView.
The functionalized starch modifies the mitochondrial surface and encapsulates resveratrol into the cavity of helices, facilitating a novel mitochondrial transplantation platform (MLSR). Upon internalization in recipient cells, MLSR triggers mitophagy and maintains positive autophagic flux through RIP1/RIP3 pathway inhibition.
Nisha Wang, Qiyang Ding, Lei Shi, Ji Xia, Shuyue Zhang, Chenxin Jian, Yixiao Yan, Xiuhua Luo, Jiarui Wang, Ming Cheng, Yiqiong Jia, Hao Tian, Wei Gao   +12 more
wiley   +1 more source