Results 31 to 40 of about 5,278 (225)
Defective nucleotide-dependent assembly and membrane fusion in Mfn2 CMT2A variants improved by Bax
Mfn2 CMT2A–associated variants located proximal to the hinge connecting its two extended helical bundles have impaired GTP-dependent assembly and mitochondrial fusion activity, which are both improved by cytosolic Bax.
Nyssa B Samanas +2 more
doaj +1 more source
Emerging functions of mammalian mitochondrial fusion and fission [PDF]
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
The MFN1 and MFN2 mitofusins promote clustering between mitochondria and peroxisomes
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
Mitofusins and OPA1 Mediate Sequential Steps in Mitochondrial Membrane Fusion [PDF]
Mitochondrial fusion requires the coordinated fusion of the outer and inner membranes. Three large GTPases—OPA1 and the mitofusins Mfn1 and Mfn2—are essential for the fusion of mammalian mitochondria.
David C. Chan +5 more
core +2 more sources
Pro-diversity mitofusins [PDF]
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
OPA1 mutation and late-onset cardiomyopathy: mitochondrial dysfunction and mtDNA instability. [PDF]
BackgroundMitochondrial fusion protein mutations are a cause of inherited neuropathies such as Charcot-Marie-Tooth disease and dominant optic atrophy.
Bers, Donald M +10 more
core +2 more sources
Mitofusins: Mighty Regulators of Metabolism [PDF]
Mitochondria are central regulators of cellular metabolism but how their function in a subset of cells affects whole-body energy balance is less understood. Two studies in this issue of Cell identify how diet-dependent modulation of mitochondrial fusion in specific neuronal circuits impact the metabolic status of an animal.
openaire +2 more sources
The INs and OUTs of mitofusins [PDF]
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
Disruption of fusion results in mitochondrial heterogeneity and dysfunction [PDF]
Mitochondria undergo continual cycles of fusion and fission, and the balance of these opposing processes regulates mitochondrial morphology. Paradoxically, cells invest many resources to maintain tubular mitochondrial morphology, when reducing both ...
Chan, David C. +2 more
core +2 more sources
Mutations in PINK1 and Parkin impair ubiquitination of Mitofusins in human fibroblasts.
PINK1 and Parkin mutations cause recessive Parkinson's disease (PD). In Drosophila and SH-SY5Y cells, Parkin is recruited by PINK1 to damaged mitochondria, where it ubiquitinates Mitofusins and consequently promotes mitochondrial fission and mitophagy ...
Aleksandar Rakovic +6 more
doaj +1 more source

