Results 11 to 20 of about 2,978 (195)

Mitofusin 2 Dysfunction and Disease in Mice and Men [PDF]

Frontiers in Physiology, 2020
A causal relationship between Mitofusin (MFN) 2 gene mutations and the hereditary axonal neuropathy Charcot-Marie-Tooth disease type 2A (CMT2A) was described over 15 years ago. During the intervening period much has been learned about MFN2 functioning in
Gerald W. Dorn
doaj   +4 more sources

Lipidomics reveals the reshaping of the mitochondrial phospholipid profile in cells lacking OPA1 and mitofusins

Journal of Lipid Research
Depletion or mutations of key proteins for mitochondrial fusion, like optic atrophy 1 (OPA1) and mitofusins 1 and 2 (Mfn 1 and 2), are known to significantly impact the mitochondrial ultrastructure, suggesting alterations of their membranes’ lipid ...
Tommaso R I Cataldi, Valerio Carelli, Cosima D Calvano   +2 more
exaly   +3 more sources

The role of Mitofusin-1 and Mitofusin-2 in periodontal disease: a comprehensive review. [PDF]

Front Oral Health
Periodontal disease is a widespread chronic inflammatory state influencing the supporting anatomy of the teeth, distinguished by oxidative stress, progressive bone loss, and tissue damage. Recent articles have highlighted the significance of mitochondrial dynamics, mainly Mitofusin-1 (MFN1) along with Mitofusin-2 (MFN2), inflammation regulation, tissue
Varma SR, Ani OHAA, Narayanan JK, Mathew A.   +3 more
europepmc   +4 more sources

Docking and stability defects in mitofusin highlight the proteasome as a potential therapeutic target

iScience, 2023
Summary: Defects in mitochondrial fusion are at the base of many diseases. Mitofusins power membrane-remodeling events via self-interaction and GTP hydrolysis.
Ira Buntenbroich, Vincent Anton, Daniel Perez-Hernandez, Tânia Simões, Felix Gaedke, Astrid Schauss, Gunnar Dittmar, Jan Riemer, Mafalda Escobar-Henriques   +8 more
doaj   +1 more source

The INs and OUTs of mitofusins [PDF]

Journal of Cell Biology, 2018
Mitofusins are outer membrane proteins essential for mitochondrial fusion. Their accepted topology posits that both N and C termini face the cytoplasm. In this issue, Mattie et al. (2018. J. Cell Biol. https://doi.org/10.1083/jcb.201611194) demonstrate instead that their C termini reside in the intermembrane space. These findings call for a revision of
Giacomello, Marta, Scorrano, Luca
openaire   +3 more sources

Mitofusin 2 but not mitofusin 1 mediates Bcl-XL-induced mitochondrial aggregation [PDF]

Journal of Cell Science, 2020
ABSTRACT Bcl-2 family proteins, as central players of the apoptotic program, participate in regulation of the mitochondrial network. Here, a quantitative live-cell fluorescence resonance energy transfer (FRET) two-hybrid assay was used to confirm the homo-/hetero-oligomerization of mitofusins 2 and 1 (MFN2 and MFN1), and also demonstrate
Mengyan Du, Si Yu, Wenhua Su, Mengxin Zhao, Fangfang Yang, Yangpei Liu, Zihao Mai, Yong Wang, Xiaoping Wang, Tongsheng Chen   +9 more
openaire   +2 more sources

Piperine Derivatives Enhance Fusion and Axonal Transport of Mitochondria by Activating Mitofusins

Chemistry, 2022
Piperine (1-piperoylpiperidine) is the major pungent component of black pepper (Piper nigrum) and exhibits a spectrum of pharmacological activities. The molecular bases for many of piperine’s biological effects are incompletely defined. We noted that the
Lihong Zhang, Xiawei Dang, Antonietta Franco, Haiyang Zhao, Gerald W. Dorn   +4 more
doaj   +1 more source

A human mitofusin 2 mutation can cause mitophagic cardiomyopathy

eLife, 2023
Cardiac muscle has the highest mitochondrial density of any human tissue, but mitochondrial dysfunction is not a recognized cause of isolated cardiomyopathy. Here, we determined that the rare mitofusin (MFN) 2 R400Q mutation is 15–20× over-represented in
Antonietta Franco, Jiajia Li, Daniel P Kelly, Ray E Hershberger, Ali J Marian, Renate M Lewis, Moshi Song, Xiawei Dang, Alina D Schmidt, Mary E Mathyer, John R Edwards, Cristina de Guzman Strong, Gerald W Dorn   +12 more
doaj   +1 more source

Mitofusins: from mitochondria to fertility

Cellular and Molecular Life Sciences, 2022
AbstractGerm cell formation and embryonic development require ATP synthesized by mitochondria. The dynamic system of the mitochondria, and in particular, the fusion of mitochondria, are essential for the generation of energy. Mitofusin1 and mitofusin2, the homologues of Fuzzy onions in yeast and Drosophila, are critical regulators of mitochondrial ...
Shanjiang Zhao, Nuo Heng, Huan Wang, Haoyu Wang, Haobo Zhang, Jianfei Gong, Zhihui Hu, Huabin Zhu   +7 more
openaire   +2 more sources

Essential lipid autacoids rewire mitochondrial energy efficiency in metabolic dysfunction‐associated fatty liver disease

Hepatology, EarlyView., 2022
Increased liver content of DHA‐derived small lipid autacoids (i.e resolvin D1 and maresin 1) associates with enhanced mitochondrial oxidative phosphorylation, fatty acid β‐oxidation and bioenergetic metabolic flux. These features provide hepatic protection from steatotic, pro‐inflammatory and fibrogenic insults.
Cristina López‐Vicario, David Sebastián, Mireia Casulleras, Marta Duran‐Güell, Roger Flores‐Costa, Ferran Aguilar, Juan José Lozano, Ingrid W. Zhang, Esther Titos, Jing X. Kang, Antonio Zorzano, Makoto Arita, Joan Clària   +12 more
wiley   +1 more source