Mitofusin 2 Exerts a Protective Role in Ischemia Reperfusion Injury Through Increasing Autophagy
Cellular Physiology and Biochemistry, 2018 Background/Aims: Autophagy is essential for maintaining cellular homeostasis and the survival of terminally differentiated cells as neurons. In this study, we aim to investigate whether mitofusin 2, a mitochondrial fusion protein, mediates autophagy in ...
Cheng Peng +12 more
doaj +3 more sources
Burst mitofusin activation reverses neuromuscular dysfunction in murine CMT2A [PDF]
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 +10 more
doaj +2 more sources
Mitofusin-mediated ER stress triggers neurodegeneration in pink1/parkin models of Parkinson's disease. [PDF]
, 2016 Mutations in PINK1 and PARKIN cause early-onset Parkinson's disease (PD), thought to be due to mitochondrial toxicity. Here, we show that in Drosophila pink1 and parkin mutants, defective mitochondria also give rise to endoplasmic reticulum (ER) stress ...
C. Jones (2411536) +8 more
core +9 more sources
Mitofusin 2 Deficiency Affects Energy Metabolism and Mitochondrial Biogenesis in MEF Cells. [PDF]
PLoS ONE, 2015 Mitofusin 2 (Mfn2), mitochondrial outer membrane protein which is involved in rearrangement of these organelles, was first described in pathology of hypertension and diabetes, and more recently much attention is paid to its functions in Charcot-Marie ...
Maria Kawalec +5 more
doaj +2 more sources
A new target for an old DUB: UCH-L1 regulates mitofusin-2 levels, altering mitochondrial morphology, function and calcium uptake [PDF]
Redox Biology, 2020 UCH-L1 is a deubiquitinating enzyme (DUB), highly abundant in neurons, with a sub-cellular localization dependent on its farnesylation state. Despite UCH-L1′s association with familial Parkinson's Disease (PD), the effects on mitochondrial bioenergetics ...
Fernanda M. Cerqueira +9 more
doaj +2 more sources
Structural dataset from microsecond-long simulations of yeast mitofusin Fzo1 in the context of membrane docking [PDF]
Data in Brief, 2019 In this work we present a novel set of possible auto-oligomerisation states of yeast protein Fzo1 in the context of membrane docking. The dataset reports atomistic models and trajectories derived from a molecular dynamics study of the yeast mitofusin ...
Astrid Brandner +4 more
doaj +2 more sources
Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants [PDF]
eLife, 2017 Missense mutations of valosin-containing protein (VCP) cause an autosomal dominant disease known as inclusion body myopathy, Paget disease with frontotemporal dementia (IBMPFD) and other neurodegenerative disorders.
Ting Zhang +4 more
doaj +2 more sources
, 2021 Mitochondrial fragmentation from defective fusion or unopposed fission contributes to many neurodegenerative diseases. Small molecule mitofusin activators reverse mitochondrial fragmentation in vitro, promising a novel therapeutic approach.
Sidney B. Williams (11306623) +7 more
core +4 more sources
Mitochondrial dysfunction and pharmacodynamics of mitofusin activation in murine Charcot-Marie-Tooth disease type 2A [PDF]
, 2022 Mitofusin (MFN) 1 and MFN2 are dynamin GTPase family mitochondrial proteins that mediate mitochondrial fusion requiring MFN conformational shifts, formation of macromolecular complexes on and between mitochondria, and GTP hydrolysis.
Zhang, Lihong +4 more
core +1 more source
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 +12 more
wiley +1 more source