Results 21 to 30 of about 507,433 (303)

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

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

TOM40 Mediates Mitochondrial Dysfunction Induced by α-Synuclein Accumulation in Parkinson's Disease. [PDF]

, 2013
Alpha-synuclein (α-Syn) accumulation/aggregation and mitochondrial dysfunction play prominent roles in the pathology of Parkinson's disease. We have previously shown that postmortem human dopaminergic neurons from PD brains accumulate high levels of ...
Adame, Anthony, Mueller, Sarina, Bender Andreas, Sarina Mueller, Koob Andrew O., Elstner Matthias, Elstner, Matthias, Desplats, Paula, Mante, Michael, Eliezer Masliah, Desplats Paula, Mueller Sarina, Spencer, Brian, Matthias Elstner, Thomas Klopstock, Masliah, Eliezer, Koob, Andrew O., Andreas Bender, Bender, Andreas, Masliah Eliezer, Anthony Adame, Klopstock Thomas, Rockenstein, Edward, Laub Christoph, Rockenstein Edward, Pham Emily, Brian Spencer, Mante Michael, Michael Mante, Klopstock, Thomas, Christoph Laub, Spencer Brian, Adame Anthony, Andrew O Koob, Paula Desplats, Emily Pham, Laub, Christoph, Pham, Emily, Edward Rockenstein   +38 more
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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
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Mitochondrial heterogeneity in diseases

Signal Transduction and Targeted Therapy, 2023
AbstractAs key organelles involved in cellular metabolism, mitochondria frequently undergo adaptive changes in morphology, components and functions in response to various environmental stresses and cellular demands. Previous studies of mitochondria research have gradually evolved, from focusing on morphological change analysis to systematic multiomics,
Long Chen, Mengnan Zhou, Hao Li, Delin Liu, Peng Liao, Yao Zong, Changqing Zhang, Weiguo Zou, Junjie Gao   +8 more
openaire   +3 more sources

Mitochondrial DNA deletions and neurodegeneration in multiple sclerosis

, 2010
Cerebral atrophy is a correlate of clinical progression in multiple sclerosis (MS). Mitochondria are now established to play a part in the pathogenesis of MS.
Turnbull, Doug M, Kim J. Krishnan, Iryna Ziabreva, Owain Howell, Graham R. Campbell, Don J. Mahad, Reynolds, Richard, Owen Howell, Mahad, Don J, Reeve, Amy K, Amy K. Reeve, Richard Reynolds, Ziabreva, Iryna, Campbell, Graham R, Lassmann, Hans, Doug M. Turnbull, Howell, Owen, Krishnan, Kim J, Hans Lassmann   +18 more
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Neuroimaging of mitochondrial disease [PDF]

Mitochondrion, 2008
Mitochondrial disease represents a heterogeneous group of genetic disorders that require a variety of diagnostic tests for proper determination. Neuroimaging may play a significant role in diagnosis. The various modalities of nuclear magnetic resonance imaging (MRI) allow for multiple independent detection procedures that can give important anatomical ...
Russell P, Saneto, Seth D, Friedman, Dennis W W, Shaw   +2 more
openaire   +2 more sources

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

Deceleration of fusion–fission cycles improves mitochondrial quality control during aging

, 2012
Mitochondrial dynamics and mitophagy play a key role in ensuring mitochondrial quality control. Impairment thereof was proposed to be causative to neurodegenerative diseases, diabetes, and cancer.
Michael Meyer-Hermann, Michael Meyer-Hermann (124166), Andreas S. Reichert (124162), Reichert Andreas S., Heinz D. Osiewacz (120841), Figge, Marc Thilo, Reichert, Andreas S, Marc Thilo Figge, Heinz D Osiewacz, Reichert, Andreas, Meyer-Hermann Michael, Meyer-Hermann, Michael (Prof. Dr.), Osiewacz Heinz D., Meyer-Hermann, Michael, Osiewacz, Heinz D., Andreas S Reichert, Figge Marc Thilo, Marc Thilo Figge (155511), Osiewacz, Heinz D   +18 more
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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
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