Results 31 to 40 of about 10,033,806 (381)

KLF-1 orchestrates a xenobiotic detoxification program essential for longevity of mitochondrial mutants

Nature Communications, 2019
Cytochrome P450 oxidases (CYPs) are enzymes that participate in the xenobiotic detoxification and their expression is enhanced in long-lived model organisms.
Marija Herholz, Estela Cepeda, Linda Baumann, Alexandra Kukat, Johannes Hermeling, Sarah Maciej, Karolina Szczepanowska, Victor Pavlenko, Peter Frommolt, Aleksandra Trifunovic   +9 more
doaj   +1 more source

Mitophagy and the therapeutic clearance of damaged mitochondria for neuroprotection [PDF]

, 2016
Mitochondria are the foremost producers of the cellular energy currency ATP. They are also a significant source of reactive oxygen species and an important buffer of intracellular calcium.
Bingol, Caviston, Chourasia, Cui, Daniel A. East, Duchen, East, East, Fei, Feng, Gatliff, Geisler, Guardia-Laguarta, Guo, Hanna, Jain, Jin, Kamat, Kawamata, Kim, Landes, Lee, Lee, Lefebvre, Lionaki, Liu, Lokireddy, Magrané, Martinez-Vicente, Martinou, Matic, Mattiazzi, Melser, Michalik, Michelangelo Campanella, Narendra, Narendra, Orr, Padman, Palomo, Pankiv, Panov, Pickrell, Quirós, Richter, Rugarli, Ryan, Sarraf, Schwarten, Shaw, Sheng, Shin, Stafa, Stoica, Strappazzon, Subramaniam, Tanti, Tsunemi, Valente, Van Humbeeck, Vives-Bauza, Wallace, Wang, Wang, Wu, Zhang, Zhu   +66 more
core   +1 more source

Integrative omics approaches provide biological and clinical insights: examples from mitochondrial diseases.

Journal of Clinical Investigation, 2020
High-throughput technologies for genomics, transcriptomics, proteomics, and metabolomics, and integrative analysis of these data, enable new, systems-level insights into disease pathogenesis.
Sofia Khan, Gulayse Ince-Dunn, A. Suomalainen, L. Elo   +3 more
semanticscholar   +1 more source

microRNA-mediated differential expression of TRMU, GTPBP3 and MTO1 in cell models of mitochondrial-DNA diseases

Scientific Reports, 2017
Mitochondrial diseases due to mutations in the mitochondrial (mt) DNA are heterogeneous in clinical manifestations but usually include OXPHOS dysfunction. Mechanisms by which OXPHOS dysfunction contributes to the disease phenotype invoke, apart from cell
Salvador Meseguer, Olga Boix, Carmen Navarro-González, Magda Villarroya, Rachid Boutoual, Sonia Emperador, Elena García-Arumí, Julio Montoya, M.-Eugenia Armengod   +8 more
doaj   +1 more source

Novel ELAC2 Mutations in Individuals Presenting with Variably Severe Neurological Disease in the Presence or Absence of Cardiomyopathy

Life, 2023
Transcription of mitochondrial DNA generates long polycistronic precursors whose nucleolytic cleavage yields the individual mtDNA-encoded transcripts. In most cases, this cleavage occurs at the 5′- and 3′-ends of tRNA sequences by the concerted action of
Cérane Cafournet, Sofia Zanin, Anne Guimier, Marie Hully, Zahra Assouline, Giulia Barcia, Pascale de Lonlay, Julie Steffann, Arnold Munnich, Jean-Paul Bonnefont, Agnès Rötig, Benedetta Ruzzenente, Metodi D. Metodiev   +12 more
doaj   +1 more source

Mitochondrial diseases in North America

Neurology: Genetics, 2020
Objective To describe clinical, biochemical, and genetic features of participants with mitochondrial diseases (MtDs) enrolled in the North American Mitochondrial Disease Consortium (NAMDC) Registry.
E. Barca, Yuelin Long, V. Cooley, R. Schoenaker, V. Emmanuele, S. Dimauro, B. Cohen, A. Karaa, G. Vladutiu, R. Haas, J. V. Van Hove, F. Scaglia, S. Parikh, J. Bedoyan, Susanne D DeBrosse, R. Gavrilova, R. Saneto, G. Enns, P. Stacpoole, J. Ganesh, A. Larson, Z. Zolkipli-Cunningham, Marni J. Falk, A. Goldstein, M. Tarnopolsky, A. Gropman, Kathryn M. Camp, D. Krotoski, K. Engelstad, X. Rosales, Joshua Kriger, Johnston Grier, R. Buchsbaum, John L.P. Thompson, M. Hirano   +34 more
semanticscholar   +1 more source

Mitochondrial Disease and Anesthesia [PDF]

Journal of Inborn Errors of Metabolism and Screening, 2017
It is increasingly common for children with mitochondrial disease to undergo surgery and anesthesia. Although many different anesthetics have been used successfully for these patients, serious, unexpected complications have occurred during and following anesthetic exposure.
Hsieh, Vincent C., Krane, Elliot J., Morgan, Philip G.   +2 more
openaire   +5 more sources

Rapamycin rescues mitochondrial myopathy via coordinated activation of autophagy and lysosomal biogenesis

EMBO Molecular Medicine, 2018
The mTOR inhibitor rapamycin ameliorates the clinical and biochemical phenotype of mouse, worm, and cellular models of mitochondrial disease, via an unclear mechanism.
Gabriele Civiletto, Sukru Anil Dogan, Raffaele Cerutti, Gigliola Fagiolari, Maurizio Moggio, Costanza Lamperti, Cristiane Benincá, Carlo Viscomi, Massimo Zeviani   +8 more
doaj   +1 more source

Mitochondrial dynamics and viral infections: A close nexus. [PDF]

, 2015
Viruses manipulate cellular machinery and functions to subvert intracellular environment conducive for viral proliferation. They strategically alter functions of the multitasking mitochondria to influence energy production, metabolism, survival, and ...
Khan, Mohsin, Kim, Seong-Jun, Siddiqui, Aleem, Syed, Gulam Hussain   +3 more
core   +2 more sources

Mitochondrial Dysfunction in Aging and Diseases of Aging. [PDF]

, 2019
Mitochondria have been increasingly recognized as the important players in the aging process [...]
Haas, Richard H
core   +2 more sources