Results 21 to 30 of about 17,646 (230)

Complementation between mouse Mfn1 and Mfn2 protects mitochondrial fusion defects caused by CMT2A disease mutations [PDF]

open access: yes, 2007
Mfn2, an oligomeric mitochondrial protein important for mitochondrial fusion, is mutated in Charcot-Marie-Tooth disease (CMT) type 2A, a peripheral neuropathy characterized by axonal degeneration.
Chan, David C., Detmer, Scott A.
core   +2 more sources

Intra- and Intercellular Quality Control Mechanisms of Mitochondria

open access: yesCells, 2017
Mitochondria function to generate ATP and also play important roles in cellular homeostasis, signaling, apoptosis, autophagy, and metabolism. The loss of mitochondrial function results in cell death and various types of diseases.
Yoshimitsu Kiriyama, Hiromi Nochi
doaj   +1 more source

The Effects of Regulatory Lipids on Intracellular Membrane Fusion Mediated by Dynamin-Like GTPases

open access: yesFrontiers in Cell and Developmental Biology, 2020
Membrane fusion mediates a number of fundamental biological processes such as intracellular membrane trafficking, fertilization, and viral infection. Biological membranes are composed of lipids and proteins; while lipids generally play a structural role,
Yeojin Moon, Youngsoo Jun
doaj   +1 more source

Mitofusin-Dependent ER Stress Triggers Glial Dysfunction and Nervous System Degeneration in a Drosophila Model of Friedreich’s Ataxia

open access: yesFrontiers in Molecular Neuroscience, 2018
Friedreich’s ataxia (FRDA) is the most important recessive ataxia in the Caucasian population. It is caused by a deficit of the mitochondrial protein frataxin.
Oliver Edenharter   +2 more
doaj   +1 more source

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

open access: yes, 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   +12 more
core   +2 more sources

Mitofusins: ubiquitylation promotes fusion [PDF]

open access: yesCell Research, 2014
Mitochondrial genes including Mfn2 are at the center of many diseases, underscoring their potential as a therapeutical target. The Chen group now identified 15-oxospiramilactone as a chemical inhibitor of the mammalian deubiquitylase USP30, acting on Mfn1 and Mfn2.
openaire   +2 more sources

Mitochondrial Quality Control in Aging and Heart Failure: Influence of Ketone Bodies and Mitofusin-Stabilizing Peptides

open access: yesFrontiers in Physiology, 2019
Aim: Aging and heart failure (HF) are each characterized by increased mitochondrial damage, which may contribute to further cardiac dysfunction. Mitophagy in response to mitochondrial damage can improve cardiovascular health.
Phung N. Thai   +9 more
doaj   +1 more source

Mitofusin 2 in Macrophages Links Mitochondrial ROS Production, Cytokine Release, Phagocytosis, Autophagy, and Bactericidal Activity

open access: yesCell Reports, 2020
Summary: Mitofusin 2 (Mfn2) plays a major role in mitochondrial fusion and in the maintenance of mitochondria-endoplasmic reticulum contact sites. Given that macrophages play a major role in inflammation, we studied the contribution of Mfn2 to the ...
Juan Tur   +9 more
doaj   +1 more source

Human biallelic MFN2 mutations induce mitochondrial dysfunction, upper body adipose hyperplasia, and suppression of leptin expression

open access: yeseLife, 2017
MFN2 encodes mitofusin 2, a membrane-bound mediator of mitochondrial membrane fusion and inter-organelle communication. MFN2 mutations cause axonal neuropathy, with associated lipodystrophy only occasionally noted, however homozygosity for the p ...
Nuno Rocha   +30 more
doaj   +1 more source

Emerging functions of mammalian mitochondrial fusion and fission [PDF]

open access: yes, 2005
Mitochondria provide a myriad of services to the cell, including energy production, calcium buffering and regulation of apoptosis. How these diverse functions are coordinated among the hundreds of mitochondria in a given cell is largely unknown, but is ...
Chan, David C., Chen, Hsiuchen
core   +1 more source

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