Control of mitochondrial morphology by a human mitofusin [PDF]
Journal of Cell Science, 2001 ABSTRACT Although changes in mitochondrial size and arrangement accompany both cellular differentiation and human disease, the mechanisms that mediate mitochondrial fusion, fission and morphogenesis in mammalian cells are not understood. We have identified two human genes encoding potential mediators of mitochondrial fusion.
A, Santel, M T, Fuller
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Endoplasmic Reticulum Stress and Mitochondrial Function in Airway Smooth Muscle
Frontiers in Cell and Developmental Biology, 2020 Inflammatory airway diseases such as asthma affect more than 300 million people world-wide. Inflammation triggers pathophysiology via such as tumor necrosis factor α (TNFα) and interleukins (e.g., IL-13).
Philippe Delmotte, Gary C. Sieck
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Mitofusins: ubiquitylation promotes fusion [PDF]
Cell 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.
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A mitofusin-dependent docking ring complex triggers mitochondrial fusion in vitro
eLife, 2016 Fusion of mitochondrial outer membranes is crucial for proper organelle function and involves large GTPases called mitofusins. The discrete steps that allow mitochondria to attach to one another and merge their outer membranes are unknown.
Tobias Brandt +3 more
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Lin28A induces energetic switching to glycolytic metabolism in human embryonic kidney cells [PDF]
, 2016 Background: Loss of a cell’s capacity to generate sufficient energy for cellular functions is a key hallmark of the ageing process and ultimately leads to a variety of important age-related pathologies such as cancer, Parkinson’s disease and ...
Docherty, Craig K. +2 more
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Mitochondrial dynamics–fusion, fission, movement, and mitophagy–in neurodegenerative diseases [PDF]
, 2009 Neurons are metabolically active cells with high energy demands at locations distant from the cell body. As a result, these cells are particularly dependent on mitochondrial function, as reflected by the observation that diseases of mitochondrial ...
Alexander +42 more
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Drosophila Parkin requires PINK1 for mitochondrial translocation and ubiquitinates Mitofusin [PDF]
, 2010 Loss of the E3 ubiquitin ligase Parkin causes early onset Parkinson's disease, a neurodegenerative disorder of unknown etiology. Parkin has been linked to multiple cellular processes including protein degradation, mitochondrial homeostasis, and autophagy;
A. J. Whitworth +28 more
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Frontiers in Neuroscience, 2019 Parkinson’s disease (PD) is characterized by selective degeneration of dopaminergic (DAergic) neurons in the substantia nigra pars compacta (SNpc). α-synuclein (α-syn) is known to regulate mitochondrial function and both PINK1 and Parkin have been shown ...
Mahesh Ramalingam +2 more
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The Effect of a Novel c.820C>T (Arg274Trp) Mutation in the Mitofusin 2 Gene on Fibroblast Metabolism and Clinical Manifestation in a Patient. [PDF]
PLoS ONE, 2017 Charcot-Marie-Tooth disease type 2A (CMT2A) is an autosomal dominant axonal peripheral neuropathy caused by mutations in the mitofusin 2 gene (MFN2).
Małgorzata Beręsewicz +7 more
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Neuroprotective effects of the immunomodulatory drug FK506 in a model of HIV1-gp120 neurotoxicity. [PDF]
, 2016 BackgroundHIV-associated neurocognitive disorders (HAND) continue to be a common morbidity associated with chronic HIV infection. It has been shown that HIV proteins (e.g., gp120) released from infected microglial/macrophage cells can cause neuronal ...
Achim, Cristian +9 more
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