Results 81 to 90 of about 17,646 (230)

Mitofusins as mitochondrial anchors and tethers

Journal of Molecular and Cellular Cardiology, 2020
Mitochondria have their own genomes and their own agendas. Like their primitive bacterial ancestors, mitochondria interact with their environment and organelle colleagues at their physical interfaces, the outer mitochondrial membrane. Among outer membrane proteins, mitofusins (MFN) are increasingly recognized for their roles as arbiters of mitochondria-
openaire   +3 more sources

OPA1 requires mitofusin 1 to promote mitochondrial fusion [PDF]

Proceedings of the National Academy of Sciences, 2004
The regulated equilibrium between mitochondrial fusion and fission is essential to maintain integrity of the organelle. Mechanisms of mitochondrial fusion are largely uncharacterized in mammalian cells. It is unclear whether OPA1, a dynamin-related protein of the inner membrane mutated in autosomal dominant optic atrophy, participates in fusion or ...
Cipolat S, Martins de Brito O, Dal Zilio B, SCORRANO, LUCA   +3 more
openaire   +2 more sources

Mitochondria and the Actin Cytoskeleton in Neurodegeneration

Cytoskeleton, EarlyView.
ABSTRACT Mitochondrial dysfunction and cytoskeletal disorganization are widely recognized hallmarks of neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS).
Shivani Tuli, Preet Patel, Aneri Shethji, David Gau   +3 more
wiley   +1 more source

Drosophila Parkin requires PINK1 for mitochondrial translocation and ubiquitinates Mitofusin [PDF]

, 2010
Loss of the E3 ubiquitin ligase Parkin causes early onset Parkinson's disease, a neurodegenerative disorder of unknown etiology. Parkin has been linked to multiple cellular processes including protein degradation, mitochondrial homeostasis, and autophagy;
A. J. Whitworth, Bueler, Chu, Clark, De Vos, E. Ziviani, Exner, Fallon, Gasser, Geisler, Kim, Koshiba, Liesa, Lim, Moore, Morais, Mortiboys, Narendra, Park, Park, Popp, R. N. Tao, Sandebring, Shimura, Soubannier, Steinbrink, Tatsuta, Twig, Whitworth   +28 more
core   +2 more sources

Mitofusin 1 overexpression rescues the abnormal mitochondrial dynamics caused by the Mitofusin 2 K357T mutation in vitro

Journal of the Peripheral Nervous System, 2023
AbstractBackground and aimsMitofusin 1 (MFN1) and MFN2 are outer mitochondrial membrane fusogenic proteins regulating mitochondrial network morphology. MFN2 mutations cause Charcot‐Marie‐Tooth type 2A (CMT2A), an axonal neuropathy characterized by mitochondrial fusion defects, which in the case of a GTPase domain mutant, were rescued following wild ...
Filippos Stavropoulos, Elena Georgiou, Natasa Schiza, Shaughn Bell, Robert H. Baloh, Kleopas A. Kleopa, Irene Sargiannidou   +6 more
openaire   +2 more sources

TREM1/3 deficiency impairs tissue repair after acute kidney injury and mitochondrial metabolic flexibility in tubular epithelial cells [PDF]

, 2019
Long-term sequelae of acute kidney injury (AKI) are associated with incomplete recovery of renal function and the development of chronic kidney disease (CKD), which can be mediated by aberrant innate immune activation, mitochondrial pathology, and ...
Borrelli, Cristiana, Butter, Loes M, Claessen, Nike, Colonna, Marco, Dessing, Mark C, Florquin, Sandrine, Leemans, Jaklien C, Rampanelli, Elena, Scantlebery, Angelique M.L, Soriani, Alessandra, Tammaro, Alessandra   +10 more
core   +2 more sources

Control of mitochondrial morphology by a human mitofusin [PDF]

Journal of Cell Science, 2001
ABSTRACT Although changes in mitochondrial size and arrangement accompany both cellular differentiation and human disease, the mechanisms that mediate mitochondrial fusion, fission and morphogenesis in mammalian cells are not understood. We have identified two human genes encoding potential mediators of mitochondrial fusion.
A, Santel, M T, Fuller
openaire   +2 more sources

Regulation of Mitochondrial Dynamics and Neurodegenerative Diseases [PDF]

, 2011
Mitochondria are important cellular organelles in most metabolic processes and have a highly dynamic nature, undergoing frequent fission and fusion. The dynamic balance between fission and fusion plays critical roles in mitochondrial functions. In recent
Fujimura, Atsushi, Han, Xiao-Jian, Matsui, Hideki, Matsushita, Masayuki, Nishiki, Tei-ichi, Ohmori, Iori, Tomizawa, Kazuhito   +6 more
core   +1 more source

Role of Mitofusins and Mitophagy in Life or Death Decisions

Frontiers in Cell and Developmental Biology, 2020
Mitochondria entail an incredible dynamism in their morphology, impacting death signaling and selective elimination of the damaged organelles. In turn, by recycling the superfluous or malfunctioning mitochondria, mostly prevalent during aging, mitophagy ...
Mariana Joaquim, Mafalda Escobar-Henriques   +1 more
doaj   +1 more source

Direct Small Molecule Activation of Mitofusins [PDF]

, 2018
SummaryMitochondrial fusion is a physiological process that is regulated by mitofusins on the outer mitochondrial membrane. Conformational plasticity between anti- and pro-tethering conformations of mitofusins permits mitochondrial tethering and subsequent fusion. Here we developed a pharmacophore-based model to rationally manipulate the conformational
Zacharioudakis, Emmanouil, Biris, Nikolaos, Garner, Thomas P., Chen, Yun, Pekson, Ryan, Dhingra, Rimpy, Santulli, Gaetano, Kirshenbaum, Lorrie A., Kitsis, Richard N., Gavathiotis, Evripidis   +9 more
openaire   +1 more source