Results 11 to 20 of about 735,599 (336)

Mitochondrial Dynamics in Mitochondrial Diseases

Diseases, 2016
Mitochondria are very versatile organelles in continuous fusion and fission processes in response to various cellular signals. Mitochondrial dynamics, including mitochondrial fission/fusion, movements and turnover, are essential for the mitochondrial ...
Juan M. Suárez-Rivero, Marina Villanueva-Paz, Patricia de la Cruz-Ojeda, Mario de la Mata, David Cotán, Manuel Oropesa-Ávila, Isabel de Lavera, Mónica Álvarez-Córdoba, Raquel Luzón-Hidalgo, José A. Sánchez-Alcázar   +9 more
doaj   +3 more sources

Mitochondrial diseases [PDF]

Nature Reviews Disease Primers, 2016
Mitochondrial diseases are a group of genetic disorders that are characterized by defects in oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) and mitochondrial DNA (mtDNA) that encode structural mitochondrial proteins or proteins involved in mitochondrial function.
Gorman G. S., Chinnery P. F., DiMauro S., Hirano M., Koga Y., McFarland R., Suomalainen A., Thorburn D. R., Zeviani M., Turnbull D. M.   +9 more
  +9 more sources

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

Mitochondrial Disease and Stroke [PDF]

Stroke, 2001
It is well known that some mitochondrial disorders are responsible for ischemic cerebral infarction in young patients. Our purpose was to determine, in this prospective ongoing study, whether ischemic stroke is the only manifestation of a mitochondrial disorder in young patients.Patients aged
E, Martínez-Fernández, A, Gil-Peralta, R, García-Lozano, I, Chinchón, I, Aguilera, O, Fernández-López, J, Arenas, Y, Campos, J, Bautista   +8 more
openaire   +2 more sources

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

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

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

Inhibition of the mitochondrial pyruvate carrier protects from excitotoxic neuronal death. [PDF]

, 2017
Glutamate is the dominant excitatory neurotransmitter in the brain, but under conditions of metabolic stress it can accumulate to excitotoxic levels.
Andreyev, Alexander Y, Bloodgood, Brenda L, Buren, Caodu, Cordes, Thekla, Divakaruni, Ajit S, Fields, Jerel A, Li, Edward, Martyniuk, Kelly, Metallo, Christian M, Murphy, Anne N, Raymond, Lynn A, Reynolds, Ian J, Wallace, Martina   +12 more
core   +1 more source