Results 41 to 50 of about 2,978 (195)

Burst mitofusin activation reverses neuromuscular dysfunction in murine CMT2A

eLife, 2020
Charcot–Marie-Tooth disease type 2A (CMT2A) is an untreatable childhood peripheral neuropathy caused by mutations of the mitochondrial fusion protein, mitofusin (MFN) 2.
Antonietta Franco, Xiawei Dang, Emily K Walton, Joshua N Ho, Barbara Zablocka, Cindy Ly, Timothy M Miller, Robert H Baloh, Michael E Shy, Andrew S Yoo, Gerald W Dorn II   +10 more
doaj   +1 more source

Correcting mitochondrial fusion by manipulating mitofusin conformations [PDF]

Nature, 2016
Mitochondria are dynamic organelles that exchange contents and undergo remodelling during cyclic fusion and fission. Genetic mutations in MFN2 (the gene encoding mitofusin 2) interrupt mitochondrial fusion and cause the untreatable neurodegenerative condition Charcot-Marie-Tooth disease type 2A (CMT2A). It has not yet been possible to directly modulate
Antonietta, Franco, Richard N, Kitsis, Julie A, Fleischer, Evripidis, Gavathiotis, Opher S, Kornfeld, Guohua, Gong, Nikolaos, Biris, Ann, Benz, Nir, Qvit, Sara K, Donnelly, Yun, Chen, Steven, Mennerick, Louis, Hodgson, Daria, Mochly-Rosen, Gerald W, Dorn   +14 more
openaire   +2 more sources

A Novel ENU-Induced Mfn2 Mutation Causes Motor Deficits in Mice without Causing Peripheral Neuropathy

Biology, 2023
Mitochondrial fission and fusion are required for maintaining functional mitochondria. The mitofusins (MFN1 and MFN2) are known for their roles in mediating mitochondrial fusion.
Timothy J. Hines, Janice Bailey, Hedi Liu, Anyonya R. Guntur, Kevin L. Seburn, Samia L. Pratt, Jonathan R. Funke, Lisa M. Tarantino, Robert W. Burgess   +8 more
doaj   +1 more source

Mitophagy: Mitofusin Recruits a Mitochondrial Killer [PDF]

Current Biology, 2013
Parkin is a cytosolic ubiquitin ligase that translocates to damaged mitochondria and promotes their degradation. Recent work demonstrates that a phosphorylated form of the mitochondrial fusion protein Mitofusin 2 serves as a receptor for Parkin translocation to damaged mitochondria.
openaire   +2 more sources

Structural basis for membrane tethering by a bacterial dynamin-like pair

Nature Communications, 2018
Dynamin-like proteins (DLPs) such as the mitofusins form homotypic and heterotypic oligomers that bridge and fuse opposing membranes. Here, Liu, Noel and Low present the crystal structure of a bacterial DLP heterotypic pair, providing insights into the ...
Jiwei Liu, Jeffrey K. Noel, Harry H. Low
doaj   +1 more source

Mitochondrial fusion: Reaching the end of mitofusin’s tether [PDF]

Journal of Cell Biology, 2016
In this issue, Qi et al. (2016. J. Cell Biol. https://doi.org/10.1083/jcb.201609019) provide structural insights into the mechanisms of mitochondrial outer membrane fusion by investigating the structure of mitofusin 1 (MFN1). This work proposes a new model to explain the important and elusive process of MFN-mediated mitochondrial fusion.
Formosa, Luke E., Ryan, Michael T.
openaire   +2 more sources

Plasticity in salt bridge allows fusion-competent ubiquitylation of mitofusins and Cdc48 recognition

Life Science Alliance, 2019
Mitochondrial fusion requires an alternating salt bridge between CMT2A-associated disease residues that enable GTP hydrolysis; Fzo1 ubiquitylation then licenses post-fusion recycling by Cdc48.
Vincent Anton, Ira Buntenbroich, Ramona Schuster, Felix Babatz, Tânia Simões, Selver Altin, Gaetano Calabrese, Jan Riemer, Astrid Schauss, Mafalda Escobar-Henriques   +9 more
doaj   +1 more source

UiO‐66 metal–organic frameworks in biomedicine: From structural tunability to bioimaging, photodiagnostics, and photodynamic cancer therapy

FEBS Open Bio, EarlyView.
UiO‐66(Zr) metal–organic frameworks are chemically stable, biocompatible, and highly tunable nanomaterials. Their modular structure enables controlled drug delivery, multimodal bioimaging, and light‐activated photodynamic therapy, supporting integrated diagnostic and therapeutic (theranostic) applications in cancer and biomedical research.
Veronika Huntošová, Grigorii Rakhalskii, Miroslav Almáši   +2 more
wiley   +1 more source

Unveiling the role of mitofusin proteins in periodontal inflammation: Mitofusin-1 and Mitofusin-2 perspectives

Tanta Dental Journal
Abstract Periodontal diseases result from the complex interplay between microbial pathogens and the host immune response. Emerging evidence suggests that alterations in mitochondrial dynamics proteins, particularly Mitofusin-1 (MFN1) and Mitofusin-2 (MFN2), contribute to the pathogenesis of periodontal inflammation.
Himani Sharma, Umang Tripathi
openaire   +1 more source

Mitochondrial Dysfunction Unravels the Potential Molecular Link Between Night Shift Work‐Related Circadian Disruption and Elevated Blood Pressure in Human and Mouse Models

Advanced Science, EarlyView.
This diagram illustrates that night shift work disrupts circadian clock genes (like CLOCK, BMAL1) in both humans and mice. This disruption leads to mitochondrial dysfunction (imbalanced fusion/fission proteins) and increased oxidative stress, which is identified as the primary mechanism ultimately causing elevated blood pressure.
Zhaoqiang Jiang, Yifan Dou, Yingnan Lei, Shuang Liu, Jialin Zhou, Jiaping Li, Huadong Xu, Lingfang Feng, Yongxin Li, Chuyan Zhang, Tao Li, Jianfei Wang, Xing Zhang, Xinran Wang, Luyao Liu, Jingjing Zhang, Jianlin Lou   +16 more
wiley   +1 more source