Broad activation of the ubiquitin-proteasome system by Parkin is critical for mitophagy [PDF]
Parkin, an E3 ubiquitin ligase implicated in Parkinson's disease, promotes degradation of dysfunctional mitochondria by autophagy. Using proteomic and cellular approaches, we show that upon translocation to mitochondria, Parkin activates the ubiquitin ...
Anh H. Pham +49 more
core +3 more sources
Role of mitofusin 2 in the renal stress response. [PDF]
The role of mitofusin 2 (MFN2), a key regulator of mitochondrial morphology and function in the renal stress response is unknown. To assess its role, the MFN2 floxed gene was conditionally deleted in the kidney of mice (MFN2 cKO) by Pax2 promoter driven ...
Jonathan M Gall +8 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
Signal transducer and activator of transcription 2 deficiency is a novel disorder of mitochondrial fission [PDF]
Defects of mitochondrial dynamics are emerging causes of neurological disease. In two children presenting with severe neurological deterioration following viral infection we identified a novel homozygous STAT2 mutation, c.1836C4A (p.Cys612Ter), using ...
Anderson, G +14 more
core +1 more source
Reply: MFN2 mutations cause compensatory mitochondrial DNA proliferation [PDF]
ARTICLE Sir, We recently reported in Brain a large Tunisian family with optic atrophy ‘plus’ phenotype including axonal neuropathy, deafness, cerebellar ataxia and mitochondrial myopathy with ragged-red and cytochrome c oxidase (COX)-negative fibres (Rouzier et al ., 2012). This neurological disorder is linked to a novel heterozygous missense mutation
C. Rouzier +12 more
openaire +1 more source
The essential role of mitochondrial dynamics in antiviral immunity. [PDF]
Viruses alter cellular physiology and function to establish cellular environment conducive for viral proliferation. Viral immune evasion is an essential aspect of viral persistence and proliferation.
Ahn, Dae-Gyun +3 more
core +2 more sources
MFN2 Couples Glutamate Excitotoxicity and Mitochondrial Dysfunction in Motor Neurons* [PDF]
Mitochondrial dysfunction plays a central role in glutamate-evoked neuronal excitotoxicity, and mitochondrial fission/fusion dynamics are essential for mitochondrial morphology and function. Here, we establish a novel mechanistic linker among glutamate excitotoxicity, mitochondrial dynamics, and mitochondrial dysfunction in spinal cord motor neurons ...
Wenzhang, Wang +9 more
openaire +2 more sources
BackgroundMitochondrial dynamics play a critical role in mitochondrial function. The mitofusin 2 (MFN2) gene encodes a mitochondrial membrane protein that participates in mitochondrial fusion to maintain and operate the mitochondrial network.
Wenjun Xiong +21 more
doaj +1 more source
Evidence for the involvement of lipid rafts localized at the ER-mitochondria associated membranes in autophagosome formation [PDF]
Mitochondria-associated membranes (MAMs) are subdomains of the endoplasmic reticulum (ER) that interact with mitochondria. This membrane scrambling between ER and mitochondria appears to play a critical role in the earliest steps of autophagy.
Faggioni, Alberto +9 more
core +2 more sources
Mitofusin-2 Negatively Regulates Melanogenesis by Modulating Mitochondrial ROS Generation
Inter-organellar communication is emerging as one of the most crucial regulators of cellular physiology. One of the key regulators of inter-organellar communication is Mitofusin-2 (MFN2).
Jyoti Tanwar +12 more
doaj +1 more source

