Results 21 to 30 of about 21,491 (246)

MFN2 Deficiency Impairs Mitochondrial Transport and Downregulates Motor Protein Expression in Human Spinal Motor Neurons

Frontiers in Molecular Neuroscience, 2021
Charcot-Marie-Tooth (CMT) disease is one of the most common genetically inherited neurological disorders and CMT type 2A (CMT 2A) is caused by dominant mutations in the mitofusin-2 (MFN2) gene. MFN2 is located in the outer mitochondrial membrane and is a
Yongchao Mou, Yongchao Mou, Joshua Dein, Zhenyu Chen, Zhenyu Chen, Mrunali Jagdale, Mrunali Jagdale, Xue-Jun Li, Xue-Jun Li   +8 more
doaj   +1 more source

Disruption of fusion results in mitochondrial heterogeneity and dysfunction [PDF]

, 2005
Mitochondria undergo continual cycles of fusion and fission, and the balance of these opposing processes regulates mitochondrial morphology. Paradoxically, cells invest many resources to maintain tubular mitochondrial morphology, when reducing both ...
Chan, David C., Chen, Hsiuchen, Chomyn, Anne   +2 more
core   +2 more sources

Mitochondrial Fusion Protein Mfn2 and Its Role in Heart Failure [PDF]

Frontiers in Molecular Biosciences, 2021
Mitofusin 2 (Mfn2) is a transmembrane GTPase located on the mitochondrial outer membrane that contributes to mitochondrial network regulation. It is an essential multifunctional protein that participates in various biological processes under physical and pathological conditions, including mitochondrial fusion, reticulum–mitochondria contacts ...
Lei Chen, Bilin Liu, Yuan Qin, Yuan Qin, Anqi Li, Meng Gao, Hanyu Liu, Guohua Gong, Guohua Gong   +8 more
openaire   +3 more sources

MFN2 knockdown promotes osteogenic differentiation of iPSC-MSCs through aerobic glycolysis mediated by the Wnt/β-catenin signaling pathway

Stem Cell Research & Therapy, 2022
Background Mitofusin-2 (MFN2) is a kind of GTPase that participates in the regulation of mitochondrial fusion, which is related to a variety of physiological and pathological processes, including energy metabolism, cell differentiation, and embryonic ...
Lidi Deng, Siqi Yi, Xiaohui Yin, Yang Li, Qingxian Luan   +4 more
doaj   +1 more source

Assessing the effects of mitofusin 2 deficiency in the adult heart using 3D electron tomography [PDF]

, 2017
The effects of mitofusin 2 (MFN2) deficiency, on mitochondrial morphology and the mitochondria-junctional sarcoplasmic reticulum (jSR) complex in the adult heart, have been previously investigated using 2D electron microscopy, an approach which is unable
Cabrera-Fuentes, Hector, Chakraborty, Bibhas, Chinda, Kroekkiat, Cooper, Jackie, Dorn, Gerald W, II, Fan, Qiao, Hall, Andrew R, Hausenloy, Derek J, Hernandez-Resendiz, Sauri, Kalkhoran, Siavash Beikoghli, Ong, Sang-Bing, White, Ian J, Yellon, Derek M   +12 more
core   +2 more sources

Mitofusin 2 protects hepatocyte mitochondrial function from damage induced by GCDCA. [PDF]

PLoS ONE, 2013
Mitochondrial impairment is hypothesized to contribute to the pathogenesis of chronic cholestatic liver diseases. Mitofusin 2 (Mfn2) regulates mitochondrial morphology and signaling and is involved in the development of numerous mitochondrial-related ...
Yongbiao Chen, Lizhi Lv, Zhelong Jiang, Hejun Yang, Song Li, Yi Jiang   +5 more
doaj   +1 more source

Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function

Cell Reports, 2023
Summary: The nucleus accumbens (NAc) is a brain hub regulating motivated behaviors, including social competitiveness. Mitochondrial function in the NAc links anxiety with social competitiveness, and the mitochondrial fusion protein mitofusin 2 (Mfn2) in ...
Sriparna Ghosal, Elias Gebara, Eva Ramos-Fernández, Alessandro Chioino, Jocelyn Grosse, Isabelle Guillot de Suduiraut, Olivia Zanoletti, Bernard Schneider, Antonio Zorzano, Simone Astori, Carmen Sandi   +10 more
doaj   +1 more source

Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria. [PDF]

, 2017
SCL25A46 is a mitochondrial carrier protein that surprisingly localizes to the outer membrane and is distantly related to Ugo1. Here we show that a subset of SLC25A46 interacts with mitochondrial dynamics components and the MICOS complex.
Claypool, Steven M, Jen, Joanna C, Koehler, Carla M, Steffen, Janos, Vashisht, Ajay A, Wan, Jijun, Wohlschlegel, James A   +6 more
core   +1 more source

MFN2, a new gene responsible for mitochondrial DNA depletion [PDF]

Brain, 2012
Sir, we read with great interest the paper by Rouzier et al. (2012) reporting a novel dominant MFN2 missense mutation (c.629A>T, p.D210V) in a large family displaying an optic atrophy ‘plus’ phenotype. Importantly, the authors identified MFN2 as a new gene implicated in mitochondrial DNA instability as they found multiple mitochondrial DNA deletions in
Florence, Renaldo, Patrizia, Amati-Bonneau, Abdelhamid, Slama, Claudia, Romana, Veronique, Forin, Diane, Doummar, Christine, Barnerias, Joseph, Bursztyn, Michèle, Mayer, Nejib, Khouri, Thierry, Billette de Villemeur, Lydie, Burglen, Pascal, Reynier, Antoinette, Bernabe Gelot, Diana, Rodriguez   +14 more
openaire   +2 more sources

The Charcot–Marie Tooth Disease Mutation R94Q in MFN2 Decreases ATP Production but Increases Mitochondrial Respiration under Conditions of Mild Oxidative Stress

Cells, 2019
Charcot−Marie tooth disease is a hereditary polyneuropathy caused by mutations in Mitofusin-2 (MFN2), a GTPase in the outer mitochondrial membrane involved in the regulation of mitochondrial fusion and bioenergetics.
Christina Wolf, Rahel Zimmermann, Osamah Thaher, Diones Bueno, Verena Wüllner, Michael K.E. Schäfer, Philipp Albrecht, Axel Methner   +7 more
doaj   +1 more source