Results 111 to 120 of about 17,474 (245)

Implications of mitochondrial dynamics on neurodegeneration and on hypothalamic dysfunction [PDF]

, 2015
Mitochondrial dynamics is a term that encompasses the movement of mitochondria along the cytoskeleton, regulation of their architecture, and connectivity mediated by tethering and fusion/fission.
Antonio Zorzano, Marc Claret
core   +2 more sources

Discovery of PHB1 as a Novel Candidate Gene in Dominant Optic Atrophy

Clinical Genetics, EarlyView.
A heterozygous PHB1 missense variant (p.Ser147Phe) segregates with autosomal dominant optic atrophy in a multi‐generation family. Structural and cellular analyses suggest altered mitochondrial dynamics, identifying PHB1 as a novel candidate gene for hereditary optic neuropathy. ABSTRACT Hereditary optic neuropathies comprise a genetically heterogeneous
Marija Volk, Aleš Maver, Martina Jarc Vidmar, Nuša Trošt, Tanja Višnjar, Ana Fakin, Lea Kovač, Maja Šuštar Habjan, Lucija Malinar, Sanja Petrović Pajić, Urška Dragin Jerman, Rok Romih, Marko Hawlina, Borut Peterlin   +13 more
wiley   +1 more source

Role of mitofusin 2 (Mfn2) in controlling cellular proliferation

The FASEB Journal, 2013
It has been reported that Mitofusin2 (Mfn2) inhibits cell proliferation when overexpressed. We wanted to study the role of endogenous Mfn2 in cell proliferation, along with the structural features of Mfn2 that influence its mitochondrial localization and control of cell proliferation. Mfn2‐knockdown clones of a B‐cell lymphoma cell
Kuang-Hueih, Chen, Asish, Dasgupta, Jinhui, Ding, Fred E, Indig, Paritosh, Ghosh, Dan L, Longo   +5 more
openaire   +3 more sources

MFN2-a multifaceted guardian against Parkinson’s pathophysiology: mitochondria, ferroptosis, inflammation and oxidative stress

Frontiers in Aging Neuroscience
BackgroundParkinson’s disease (PD) is the second most prevalent neurodegenerative disease worldwide and its exact pathogenesis remains unclear. This study aims to comprehensively explore the role of MFN2 in PD based on in vivo and in vitro models for ...
Yan Cheng, Hongjiang Zhai, Yong Liu, Yunzhou Yang, Bo Fang, Mingxiang Song, Xiuqin Wang, Ping Zhong   +7 more
doaj   +1 more source

A membrane-inserted structural model of the yeast mitofusin Fzo1 [PDF]

, 2017
Mitofusins are large transmembrane GTPases of the dynamin-related protein family, and are required for the tethering and fusion of mitochondrial outer membranes.
A Fiser, A Franco, A Lupas, A Sali, A Santel, AA Polyansky, AB Knott, AM Bertholet, B Brosig, B Hess, B Westermann, C Notredame, C Volland, CR Søndergaard, D Naon, D Rapaport, DT Jones, DT Jones, E Boutet, EE Griffin, EF Pettersen, EG Hutchinson, F Anton, F Anton, F Armougom, F Sievers, G Pollastri, GJ Hermann, H Kishida, H Sesaki, H Viklund, HH Low, HH Low, HJC Berendsen, HM Berman, IM Wallace, JM Mason, JS Chappie, K Hofmann, K Labbé, L Cavellini, L Yan, L Yan, M Escobar-Henriques, M Rojo, M-P Durrieu, M-Y Shen, MA Lemmon, MA Lomize, MM Cohen, MS Longtine, N Ishihara, NJ Gleason, O Daumke, OM Brito de, P Hubert, R Filadi, R Yan, RA Laskowski, RA Laskowski, RFS Walters, RL Dunbrack, S Fritz, S Jo, S Lee, S-Q Zhang, SF Altschul, T Darden, T Koshiba, T Nugent, T Shutt, TJ Dolinsky, VB Chen, W Kabsch, WL Jorgensen, X Bian, X Daura, Y Qi, Y-L Cao   +78 more
core   +4 more sources

ER proteostasis meets mitochondrial function: contact sites as hubs of communication and therapeutic targets

The FEBS Journal, EarlyView.
Proteostasis ensures proper protein folding, modification, and degradation, while its impairment triggers ER stress. Chronic ER stress and maladaptive UPR via the CHOP–ERO1 axis remodel ERMCs, altering calcium signaling and mitochondrial metabolism.
Giorgia Maria Renna, Alessandro Cherubini, Ersilia Varone, Serena Germani, Alice Marrazza, Ester Zito   +5 more
wiley   +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

Inhibition of cAMP/PKA Pathway Protects Optic Nerve Head Astrocytes against Oxidative Stress by Akt/Bax Phosphorylation-Mediated Mfn1/2 Oligomerization. [PDF]

, 2019
Glaucoma is characterized by a progressive optic nerve degeneration and retinal ganglion cell loss, but the underlying biological basis for the accompanying neurodegeneration is not known.
Ahn, Sangphil, Edwards, Genea, Ju, Won-Kyu, Kim, Keun-Young, Park, Tae Lim, Shim, Myoung Sup, Weinreb, Robert N   +6 more
core   +1 more source

Structural analysis of a trimeric assembly of the mitochondrial dynamin-like GTPase Mgm1. [PDF]

, 2020
The fusion of inner mitochondrial membranes requires dynamin-like GTPases, Mgm1 in yeast and OPA1 in mammals, but how they mediate membrane fusion is poorly understood.
Hu, Junjie, Li, Lei, Lou, Zhiyong, Nunnari, Jodi, Qi, Yuanbo, Rao, Zihe, Ricketson, Derek, Sarsam, Reta, Subramanian, Kelly, Wong, Melissa, Wu, Lijie, Yan, Liming, Yu, Caiting, Zhao, Jinghua   +13 more
core  

Signalling Mechanisms Underlying Doxorubicin and Nox2 NADPH Oxidase-Induced Cardiomyopathy: Involvement of Mitofusin-2 [PDF]

, 2017
BACKGROUND AND PURPOSE: The anthracycline doxorubicin (DOX), although successful as a first-line cancer treatment, induces cardiotoxicity linked with increased production of myocardial reactive oxygen species (ROS), with Nox2 NADPH oxidase-derived ...
Dunne, Philip D, Edgar, Kevin S, Grieve, David J, Kearney, Anna M, McDermott, Barbara J, McLaughlin, Declan, O'Neill, Karla M, Zhao, Youyou   +7 more
core   +1 more source