Results 51 to 60 of about 12,758 (225)
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.
openaire +2 more sources
Complementation of mitofusin-2 KO cells. [PDF]
, 2013 Mfn2 KO cells were transfected with a plasmid driving the expression of Mitofusin-2 fused to GFP. Cells expressing moderate levels of fluorescence were sorted, re-grown, fixed and processed as described in the legend to Fig. 1.
Pierre Cosson (290984) +3 more
core +1 more source
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
doaj +1 more source
Functional characterization of the membrane bound E3 ligase MARCH5 and its substrate Mitofusin-2 [PDF]
, 2022 Mitochondrial integrity relies on balanced fission and fusion events. The ubiquitination, mediated by the mitochondrial membrane-bound ubiquitin ligase MARCH5, is crucial for the regulation of these mitochondrial dynamics by targeting proteins of the ...
Merklinger, Lisa
core
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
doaj +1 more source
Mitofusins as mitochondrial anchors and tethers
Journal of Molecular and Cellular Cardiology, 2020 Mitochondria have their own genomes and their own agendas. Like their primitive bacterial ancestors, mitochondria interact with their environment and organelle colleagues at their physical interfaces, the outer mitochondrial membrane. Among outer membrane proteins, mitofusins (MFN) are increasingly recognized for their roles as arbiters of mitochondria-
openaire +3 more sources
Pro-diversity mitofusins [PDF]
The Journal of Cell Biology, 2007 Affirmative action groups would be proud of Mfn proteins. These fusion proteins work better with diverse partners, say Detmer and Chan (page [405][1]). The results might help explain why only certain neurons are susceptible in Charcot-Marie-Tooth disease 2A (CMT2A).
openaire +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 +14 more
openaire +2 more sources
The MFN1 and MFN2 mitofusins promote clustering between mitochondria and peroxisomes
Communications Biology, 2022 The MFN1 and MFN2 mitofusin proteins localize to the outer mitochondrial membrane, where they may help functionally tether mitochondria to peroxisomes.
Yinbo Huo +4 more
doaj +1 more source
Mice Hemizygous for a Pathogenic Mitofusin-2 Allele Exhibit Hind Limb/Foot Gait Deficits and Phenotypic Perturbations in Nerve and Muscle. [PDF]
PLoS ONE, 2016 Charcot-Marie-Tooth disease type 2A (CMT2A), the most common axonal form of hereditary sensory motor neuropathy, is caused by mutations of mitofusin-2 (MFN2). Mitofusin-2 is a GTPase required for fusion of mitochondrial outer membranes, repair of damaged
Peter Bannerman +4 more
doaj +1 more source