Results 91 to 100 of about 17,663,383 (215)

Mitofusin 2 keeps the respiratory chain on Q [PDF]

Journal of Cell Biology, 2015
![Figure][1] Many mitochondria (green) show reduced membrane potential (indicated by the potentiometric reporter TMRM, red) in fibroblasts lacking MFN2. [Mourier et al.][2] reveal that the mitochondrial fusion protein Mitofusin 2 (MFN2) is required to maintain production of the ...
openaire   +1 more source

Charcot-Marie-tooth disease type 2A: An update on pathogenesis and therapeutic perspectives

Neurobiology of Disease
Mutations in the gene encoding MFN2 have been identified as associated with Charcot–Marie–Tooth disease type 2A (CMT2A), a neurological disorder characterized by a broad clinical phenotype involving the entire nervous system.
Claudia Alberti, Federica Rizzo, Alessia Anastasia, Giacomo Comi, Stefania Corti, Elena Abati   +5 more
doaj   +1 more source

Mitofusin 2 Is Essential for IP3-Mediated SR/Mitochondria Metabolic Feedback in Ventricular Myocytes

Frontiers in Physiology, 2019
Aim: Endothelin-1 (ET-1) and angiotensin II (Ang II) are multifunctional peptide hormones that regulate the function of the cardiovascular and renal systems.
Lea K. Seidlmayer, Lea K. Seidlmayer, Christine Mages, Christine Mages, Annette Berbner, Petra Eder-Negrin, Paula Anahi Arias-Loza, Mathias Kaspar, Moshi Song, Gerald W. Dorn, Michael Kohlhaas, Stefan Frantz, Stefan Frantz, Christoph Maack, Brenda Gerull, Elena N. Dedkova, Elena N. Dedkova   +16 more
doaj   +1 more source

Canonical and non‐canonical functions of proteins regulating mitochondrial dynamics in mammalian physiology

The Journal of Physiology, EarlyView.
Abstract figure legend Mitochondria are highly dynamic organelles that continuously remodel their architecture through coordinated cycles of fusion and fission. This review examines the four key GTPases that orchestrate mitochondrial dynamics in mammals: MFN1, MFN2, OPA1, and DRP1.
Rémi Chaney, Vincent Marcangeli, Krystel Desjardins, Shima Taherkhani, Gilles Gouspillou   +4 more
wiley   +1 more source

Small Molecule Activators of Mitochondrial Fusion Prevent Congenital Heart Defects Induced by Maternal Diabetes

JACC: Basic to Translational Science
Summary: Most congenital heart defect (CHD) cases are attributed to nongenetic factors; however, the mechanisms underlying nongenetic factor–induced CHDs are elusive.
Guanglei Wang, PhD, Wenhui Lu, PhD, Wei-Bin Shen, PhD, Mariusz Karbowski, PhD, Sunjay Kaushal, MD, PhD, Peixin Yang, PhD   +5 more
doaj   +1 more source

Mitochondrial oxidative stress, calcium and dynamics in cardiac ischaemia‐reperfusion injury

The Journal of Physiology, EarlyView.
Abstract figure legend Heart attack causes ischaemia–reperfusion injury in cardiomyocytes. Mitochondria generate reactive oxygen species (ROS), leading to oxidative stress. High levels of mitochondrial calcium (Ca2+) activate the mitochondrial permeability transition pore (mPTP), and excess ROS levels can lower the Ca2+ required to activate the mPTP ...
Emily Rozich, Ulas Ozkurede, Shanmugasundaram Pakkiriswami, Ryan Gemilere, Samira M. Azarin, Julia C. Liu   +5 more
wiley   +1 more source

Mitochondrial dysfunction and pharmacodynamics of mitofusin activation in murine Charcot-Marie-Tooth disease type 2A

, 2022
Mitofusin (MFN) 1 and MFN2 are dynamin GTPase family mitochondrial proteins that mediate mitochondrial fusion requiring MFN conformational shifts, formation of macromolecular complexes on and between mitochondria, and GTP hydrolysis.
Zhang, Lihong, Molinoff, Perry B, Dorn, Gerald W, 2nd, Franco, Antonietta, Dang, Xiawei   +4 more
core   +1 more source

Placental mitochondrial metabolic adaptation maintains cellular energy balance in pregnancy complicated by gestational hypoxia

The Journal of Physiology, EarlyView.
Abstract figure legend Placental mitochondrial adaptation to gestational hypoxia. Hypoxic pregnancy in sheep increases placental insulin like growth factor 2 (IGF2) signalling (1), which is associated with a shift in capacity away from β‐oxidation (2) and complex I‐mediated respiration (3), while maintaining total oxidative phosphorylation capacity (4).
Wen Tong, Beth J. Allison, Kirsty L. Brain, Olga V. Patey, Youguo Niu, Kimberley J. Botting, Sage G. Ford, Tess A. Garrud, Peter F. B. Wooding, Qiang Lyu, Lin Zhang, Jin Ma, Alice P. Sowton, Katie A. O'Brien, Tereza Cindrova‐Davies, Hong Wa Yung, Graham J. Burton, Andrew J. Murray, Dino A. Giussani   +18 more
wiley   +1 more source

Methods to Study Mitochondrial Metabolism and Homeostasis in Fission Yeast

Yeast, EarlyView.
Ferran Gómez‐Armengol, Elena Hidalgo, Montserrat Vega   +2 more
wiley   +1 more source

Mitochondrial control of ciliary gene expression and structure in striatal neurons

The Journal of Physiology, EarlyView.
Abstract figure legend Neurons drive animal behaviour by receiving and transmitting information and require energy, primarily supplied by mitochondria, to function. Additionally, neurons need to sense environmental changes to adapt, a function that is locally played by the primary cilia.
Dogukan H. Ulgen, Alessandro Chioino, Olivia Zanoletti, Albert Quintana, Elisenda Sanz, Carmen Sandi   +5 more
wiley   +1 more source