Results 31 to 40 of about 10,435,022 (365)

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

Physical Exercise and Mitochondrial Disease: Insights From a Mouse Model

Frontiers in Neurology, 2019
Purpose: Mitochondrial diseases (MD) are among the most prevalent neuromuscular disorders. Unfortunately, no curative treatment is yet available. This study analyzed the effects of exercise training in an animal model of respiratory chain complex I ...
Carmen Fiuza-Luces, Pedro L. Valenzuela, Sara Laine-Menéndez, Miguel Fernández-de la Torre, Verónica Bermejo-Gómez, Laura Rufián-Vázquez, Joaquín Arenas, Joaquín Arenas, Miguel A. Martín, Miguel A. Martín, Alejandro Lucia, Alejandro Lucia, María Morán, María Morán   +13 more
doaj   +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

Mitochondria-originated redox signalling regulates KLF-1 to promote longevity in Caenorhabditis elegans

Redox Biology, 2022
Alternations of redox metabolism have been associated with the extension of lifespan in roundworm Caenorhabditis elegans, caused by moderate mitochondrial dysfunction, although the underlying signalling cascades are largely unknown.
Johannes CW Hermeling, Marija Herholz, Linda Baumann, Estela Cepeda Cores, Aleksandra Zečić, Thorsten Hoppe, Jan Riemer, Aleksandra Trifunovic   +7 more
doaj   +1 more source

Human mitochondrial degradosome prevents harmful mitochondrial R loops and mitochondrial genome instability [PDF]

, 2018
R loops are nucleic acid structures comprising an DNA-RNA hybrid and a displaced single-stranded DNA. These structures may occur transiently during transcription, playing essential biological functions. However, persistent R loops may become pathological
Aguilera López, Andrés, Pérez de Camino Cantos, Dolores, Silva, Sonia   +2 more
core   +1 more source

Mitochondrial Disease [PDF]

Journal of Child Neurology, 2014
Mitochondrial disease was chosen as the topic for the 13th Neurobiology of Disease in Children Symposium, held on October 31, 2013, in Austin, Texas. Program codirectors Drs Bruce Cohen and William Copeland prepared an engaging agenda that included individual presentations as well as a panel discussion from leaders working to advance the field of ...
Ben Thornton, Bruce Cohen, William Copeland, Bernard L. Maria   +3 more
openaire   +2 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

Therapeutic Approaches to Treat Mitochondrial Diseases: “One-Size-Fits-All” and “Precision Medicine” Strategies

Pharmaceutics, 2020
Primary mitochondrial diseases (PMD) refer to a group of severe, often inherited genetic conditions due to mutations in the mitochondrial genome or in the nuclear genes encoding for proteins involved in oxidative phosphorylation (OXPHOS).
E. Bottani, C. Lamperti, A. Prigione, V. Tiranti, N. Persico, D. Brunetti   +5 more
semanticscholar   +1 more source

Changes in neuronal CycD/Cdk4 activity affect aging, neurodegeneration, and oxidative stress. [PDF]

, 2015
Mitochondrial dysfunction has been implicated in human diseases, including cancer, and proposed to accelerate aging. The Drosophila Cyclin-dependent protein kinase complex cyclin D/cyclin-dependent kinase 4 (CycD/Cdk4) promotes cellular growth by ...
de la Cruz, Aida Flor A, Edgar, Bruce A, Icreverzi, Amalia, Walker, David W   +3 more
core   +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