Results 31 to 40 of about 2,946,385 (264)

Emergence of the mitochondrial reticulum from fission and fusion dynamics [PDF]

, 2012
Mitochondria form a dynamic tubular reticulum within eukaryotic cells. Currently, quantitative understanding of its morphological characteristics is largely absent, despite major progress in deciphering the molecular fission and fusion machineries ...
Michael Meyer-Hermann, Michael Meyer-Hermann (124166), Andreas S. Reichert (124162), Sukhorukov, Valerii M., Daniel Dikov (124160), Valerii M. Sukhorukov (124158), Reichert, Andreas, Valerii M. Sukhorukov, Dikov, Daniel, Andreas S. Reichert, Meyer-Hermann, Michael (Prof. Dr.), Valerii M Sukhorukov, Daniel Dikov, Andreas S Reichert   +13 more
core   +1 more source

Experimental Relocation of the Mitochondrial ATP9 Gene to the Nucleus Reveals Forces Underlying Mitochondrial Genome Evolution [PDF]

, 2012
Only a few genes remain in the mitochondrial genome retained by every eukaryotic organism that carry out essential functions and are implicated in severe diseases.
Tetaud, Emmanuel, Clauder-Münster, Sandra, di Rago, Jean-Paul, Giraud, Marie-France, Salin, Bénédicte, Godard, Franc¸ois, Sagot, Isabelle, Hermann-Le Denmat, Sylvie, Martos, Alexandre, Steinmetz, Lars M., Sellem, Carole H, Sainsard-Chanet, Annie, Contamine, Veronique, Bietenhader, Mailis, Kucharczyk, Roza, Aiyar, Raeka S, Dequard-Chablat, Michelle, Gagneur, Julien   +17 more
core   +2 more sources

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

Application of Genome Sequencing from Blood to Diagnose Mitochondrial Diseases

, 2021
Mitochondrial diseases can be caused by pathogenic variants in nuclear or mitochondrial DNA-encoded genes that often lead to multisystemic symptoms and can have any mode of inheritance. Using a single test, Genome Sequencing (GS) can effectively identify
Rocio Rius, Alison G. Compton, Minoche, Andre E, AnneMarie E. Welch, Andre E. Minoche, Coman, David, Thorburn, David R, Cowley, Mark J, David Coman, Maina P. Kava, Naomi L. Baker, Welch, AnneMarie E, Rius, Rocio, Compton, Alison G, John Christodoulou, Baker, Naomi L, Christodoulou, John, David R. Thorburn, Mark J. Cowley, Kava, Maina P   +19 more
core   +1 more source

Clinically translatable mitochondrial gene therapy in muscle using tandem mtZFN architecture

EMBO Molecular Medicine
Mutations in the mitochondrial genome (mtDNA) often lead to clinical pathologies. Mitochondrially-targeted zinc finger nucleases (mtZFNs) have been successful in reducing the levels of mutation-bearing mtDNA both in vivo and in vitro, resulting in a ...
Pavel A Nash, Keira M Turner, Christopher A Powell, Lindsey Van Haute, Pedro Silva-Pinheiro, Felix Bubeck, Ellen Wiedtke, Eloïse Marques, Dylan G Ryan, Dirk Grimm, Payam A Gammage, Michal Minczuk   +11 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

Gut microbiome and aging—A dynamic interplay of microbes, metabolites, and the immune system

FEBS Letters, EarlyView.
Age‐dependent shifts in microbial communities engender shifts in microbial metabolite profiles. These in turn drive shifts in barrier surface permeability of the gut and brain and induce immune activation. When paired with preexisting age‐related chronic inflammation this increases the risk of neuroinflammation and neurodegenerative diseases.
Aaron Mehl, Eran Blacher
wiley   +1 more source

Recombinant Mitochondrial Transcription Factor A with N-terminal Mitochondrial Transduction Domain Increases Respiration and Mitochondrial Gene Expression in G11778A Leber's Hereditary Optic Neuropathy Cybrid Cells [PDF]

, 2008
Diseases involving mitochondrial defects usually manifest themselves in high-energy, post-mitotic tissues such as brain, retina, skeletal and cardiac muscle and frequently cause deficiencies in mitochondrial bioenergetics.
James P. Bennett, Ravindar R. Thomas, Shilpa Iyer, Francisco R. Portell, Caitlin K. Quigley, Lisa D. Dunahm   +5 more
core  

Maternal age effect and severe germ-line bottleneck in the inheritance of human mitochondrial DNA [PDF]

, 2014
The manifestation of mitochondrial DNA (mtDNA) diseases depends on the frequency of heteroplasmy (the presence of several alleles in an individual), yet its transmission across generations cannot be readily predicted owing to a lack of data on the size ...
Chiaromonte, Francesca, T. S. Korneliussen, D. Blankenberg, Blankenberg, Daniel, Korneliussen, Thorfinn Sand, I. M. Paul, Paul, IM, Holland, Mitchell M, Su, Marcia Shu-Wei, B. Rebolledo-Jaramillo, Su, MS-W, B. Dickins, A. Nekrutenko, McElhoe, Jennifer A., R. Nielsen, N. Stoler, Nielsen, Rasmus, Nekrutenko, A, Makova, Kateryna D., Stoler, Nicholas, McElhoe, Jennifer A, Korneliussen, Thorfinn S., Chiaromonte, F, Holland, MM, M. S.-W. Su, Nekrutenko, Anton, Nielsen, R, Makova, KD, J. A. McElhoe, Rebolledo-Jaramillo, Boris, Rebolledo-Jaramillo, B, Korneliussen, Thorfinn S, Makova, Kateryna D, M. M. Holland, Holland, Mitchell M., Stoler, N, Blankenberg, D, F. Chiaromonte, Paul, Ian M., Paul, Ian M, K. D. Makova, Korneliussen, TS, McElhoe, JA, Dickins, Benjamin, Dickins, B   +44 more
core   +1 more source

Mitochondrial CISD1/Cisd accumulation blocks mitophagy and genetic or pharmacological inhibition rescues neurodegenerative phenotypes in Pink1/parkin models

Molecular Neurodegeneration
Background Mitochondrial dysfunction and toxic protein aggregates have been shown to be key features in the pathogenesis of neurodegenerative diseases, such as Parkinson’s disease (PD).
Aitor Martinez, Alvaro Sanchez-Martinez, Jake T. Pickering, Madeleine J. Twyning, Ana Terriente-Felix, Po-Lin Chen, Chun-Hong Chen, Alexander J. Whitworth   +7 more
doaj   +1 more source