Results 81 to 90 of about 5,278 (225)

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

Functional wiring of proteostatic and mitostatic modules ensures transient organismal survival during imbalanced mitochondrial dynamics

Redox Biology, 2019
Being an assembly of protein machines, cells depend on adequate supply of energetic molecules for retaining their homeodynamics. Consequently, mitochondria functionality is ensured by quality control systems and mitochondrial dynamics (fusion/fission ...
Sentiljana Gumeni, Zoi Evangelakou, Eleni N. Tsakiri, Luca Scorrano, Ioannis P. Trougakos   +4 more
doaj   +1 more source

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

The abundance of the ARL2 GTPase and its GAP, ELMOD2, at mitochondria are modulated by the fusogenic activity of mitofusins and stressors. [PDF]

PLoS ONE, 2017
Mitochondria are essential, dynamic organelles that respond to a number of stressors with changes in morphology that are linked to several mitochondrial functions, though the mechanisms involved are poorly understood.
Laura E Newman, Cara R Schiavon, Chengjing Zhou, Richard A Kahn   +3 more
doaj   +1 more source

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

Dual role of a GTPase conformational switch for membrane fusion by mitofusin ubiquitylation

Life Science Alliance, 2020
Mitochondrial fusion requires a conformational change in α4 of the GTPase domain, followed by AAA-ATPase regulation, thus revealing striking mechanistic similarities between small and large GTPases. Mitochondria are essential organelles whose function is
Ramona Schuster, Vincent Anton, Tânia Simões, Selver Altin, Fabian den Brave, Thomas Hermanns, Manuela Hospenthal, David Komander, Gunnar Dittmar, R Jürgen Dohmen, Mafalda Escobar-Henriques   +10 more
doaj   +1 more source

LncRNA ANRIL mediates endothelial dysfunction through BDNF downregulation in chronic kidney disease

Cell Death and Disease, 2022
Endothelial dysfunction is common in patients with chronic kidney disease (CKD), but the mechanism is unknown. In this study, we found that the circulating ANRIL level was increased and correlated with vascular endothelial dysfunction in patients with ...
Hong Su, Bing Liu, Huimin Chen, Tingwei Zhang, Tongtong Huang, Yue Liu, Cheng Wang, Qiqi Ma, Qianhui Wang, Zhimei Lv, Rong Wang   +10 more
doaj   +1 more source

Mitophagy, Mitochondrial Homeostasis, and Cell Fate

Frontiers in Cell and Developmental Biology, 2020
Mitochondria are highly plastic and dynamic organelles that have graded responses to the changing cellular, environmental, and developmental cues. Mitochondria undergo constant mitochondrial fission and fusion, mitochondrial biogenesis, and mitophagy ...
Kaili Ma, Guo Chen, Wenhui Li, Oliver Kepp, Oliver Kepp, Yushan Zhu, Quan Chen   +6 more
doaj   +1 more source