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Enhancing the profusion gene mitofusin/marf is beneficial in an in vivo model of TDP-43 (show TARDBP ELISA Kits) proteinopathies, serving as a potential therapeutic target.
activation of endoplasmic reticulum stress by defective mitochondria is neurotoxic in pink1 and parkin (show PARK2 ELISA Kits) flies and that the reduction of this signalling is neuroprotective, independently of defective mitochondria.
Clu (show CLU ELISA Kits) is upstream of and binds to VCP (show vcp ELISA Kits) in vivo and promotes VCP (show vcp ELISA Kits)-dependent Marf degradation in vitro Marf accumulates in whole muscle lysates of clu (show CLU ELISA Kits)-deficient flies and is destabilized upon Clu (show CLU ELISA Kits) overexpression. Thus, Clu (show CLU ELISA Kits) is essential for mitochondrial homeostasis and functions in concert with Parkin (show PARK2 ELISA Kits) and VCP (show vcp ELISA Kits) for Marf degradation to promote damaged mitochondrial clearance.
lack of ChChd3 leads to inactivation of Hippo activity under normal development, which is also dependent on the transcriptional coactivator Yorkie (Yki (show YAP1 ELISA Kits)). Furthermore, loss of ChChd3 induces oxidative stress and activates the JNK (show MAPK8 ELISA Kits) pathway. In addition, depletion of other mitochondrial fusion components, Opa1 (show OPA1 ELISA Kits) or Marf, inactivates the Hippo pathway as well.
Marf is required for mitochondrial fusion and transport in long axons.
Expression of Mfn2 and endoplasmic reticulum (ER) stress reduction in flies lacking Marf corrected ER shape, attenuating the developmental and motor defects.
Parkin (show PARK2 ELISA Kits) deficiency and resulting mitophagic disruption produces cardiomyopathy which can be contained by suppressing mitofusin.
mfn2 mutations alter mitochondrial dynamics and induce retinal and cardiac pathology
Data report here that Drosophila Reaper can induce mitochondrial fragmentation by binding to and inhibiting the pro-fusion protein MFN2 and its Drosophila counterpart dMFN/Marf.
MARF and Opa1 (show OPA1 ELISA Kits) control mitochondrial and cardiac function in Drosophila.
MFN2 expression was lower in patients with heart failure with preserved ejection fraction compared to controls.
Mitofusin 2 - one of a few proteins involved in a maintenance of an appropriate mitochondrial architecture, and in the consequence in the regulation of mitochondrial metabolism and calcium signalling, the controlling of the mitochondrial DNA level, and the regulation of cell proliferation and differentiation is the focus. [REVIEW]
Our patient with MFN2-related CMT2 expands the clinical and mutational spectrum of individuals with autosomal recessive CMT2 and identifies a new clinical feature that warrants further observation.
It has been shown that mitofusin-2 is modified with K6-linked polyubiquitin in a HUWE1-dependent manner.
Studied association of genetic variants of the MAVS, MITA and MFN2 genes with leprosy in Han Chinese from Southwest China; found no association between the variants and susceptibility to leprosy.
MFN2 gene polymorphisms (rs873457, rs2336384, rs1474868, rs4846085 and rs2236055) may be associated with acute liver failure and the rs873457 and rs4846085 polymorphisms are correlated with the risk and prognosis of acute liver failure.
SLC25A46 (show SLC25A46 ELISA Kits) is a new component in mitochondrial dynamics that serves as a regulator for MFN1 (show MFN1 ELISA Kits)/2 oligomerization.
Presenilin 2 (PS2 (show PSEN2 ELISA Kits)), mutations in which underlie familial Alzheimer's disease (FAD (show BRCA2 ELISA Kits)), promotes endoplasmic reticulum-mitochondria coupling only in the presence of mitofusin 2 (Mfn2).
PGC-1alpha (show PPARGC1A ELISA Kits) enhances Mfn2 transcription, but also leads to increased degradation of the Mfn2 protein, a key ubiquitylation target of Parkin (show PARK2 ELISA Kits) on mitochondria. In vivo, Parkin (show PARK2 ELISA Kits) has significant protective effects on the survival and function of nigral dopaminergic neurons in which the chronic expression of PGC-1alpha (show PPARGC1A ELISA Kits) is induced
Exome sequencing identified MFN2 SNVs in two of the individuals. Neuropathy-associated CNV outside of the PMP22 (show PMP22 ELISA Kits) locus is rare in Charcot-Marie-Tooth (CMT) disease . Nevertheless, there is potential clinical utility in testing for CNVs and exome sequencing in CMT cases negative for the CMT1A (show PMP22 ELISA Kits) duplication.
Despite apparent mitochondrial dysfunction, hearts deficient in both Mfn1 (show MFN1 ELISA Kits) and Mfn2 are protected against acute myocardial infarction due to impaired mitochondria/sarcoplasmic reticulum tethering.
Presenilin 2 (PS2 (show PSEN2 ELISA Kits)), mutations in which underlie familial Alzheimer's disease (FAD (show FANCD2 ELISA Kits)), promotes endoplasmic reticulum-mitochondria coupling only in the presence of mitofusin 2 (Mfn2).
The data of this study suggest that post-translational modification of Mfn2 is associated with its dysregulation during a window of metabolic vulnerability that precedes glaucomatous degeneration.
Study demonstrated that deregulation of mfn2 played a critical role in the mitochondrial disorder during the progression of Alzheimer's disease, and its decreased expression was regulated at least in part by miR (show MLXIP ELISA Kits)-195. Therefore, upregulation of mfn2 expression by decreasing the level of miR (show MLXIP ELISA Kits)-195 might be a potential new therapeutic strategy for treatment of Alzheimer's disease.
Mfn2 downregulation or the exogenous expression of normal Parkin (show PARK2 ELISA Kits) restored cytosolic Ca(2 (show CA2 ELISA Kits)+) transients in fibroblasts from patients with PARK2 (show PARK2 ELISA Kits) mutations, a catalytically inactive Parkinson's disease (PD)-related Parkin (show PARK2 ELISA Kits) variant had no effect. Parkin (show PARK2 ELISA Kits) is directly involved in regulating ER-mitochondria contacts and provide new insight into the role of the loss of Parkin (show PARK2 ELISA Kits) function in PD development.
Altogether, these results demonstrate that Mfn2 is a mediator of mitochondria to lipid droplet interactions, influencing lipolytic processes and whole-body energy homeostasis.
we demonstrated that by modulating mitochondrial energy metabolism through Mfn2 and mitochondrial Ca2 (show CA2 ELISA Kits)+, PPAR-b (show PPARD ELISA Kits) took an important role in neuronal differentiation induced by flavonoid compound 4a
Furthermore, analysis of muscle Mfn2-deficient mice revealed that aging-induced Mfn2 decrease underlies the age-related alterations in metabolic homeostasis and sarcopenia.
Mice hemizygous for a pathogenic Mfn2 allele exhibit hind limb/foot gait deficits and phenotypic perturbations in nerve and muscle.
MFN2 mutation status should be investigated in patients presenting with early-onset recessively inherited axonal CMT
These results highlight the essential role of mitofusin 2 in the motor axon development and demonstrate the potential of zebrafish as a suitable and complementary platform for dissecting pathogenetic mechanisms of MFN2 mutations in vivo.
A highly unusual splicing defect, where an exonic single base exchange leads to the retention of the preceding intron, was identified in MFN2 (show MFN1 ELISA Kits).
This gene encodes a mitochondrial membrane protein that participates in mitochondrial fusion and contributes to the maintenance and operation of the mitochondrial network. This protein is involved in the regulation of vascular smooth muscle cell proliferation, and it may play a role in the pathophysiology of obesity. Mutations in this gene cause Charcot-Marie-Tooth disease type 2A2, and hereditary motor and sensory neuropathy VI, which are both disorders of the peripheral nervous system. Defects in this gene have also been associated with early-onset stroke. Two transcript variants encoding the same protein have been identified.
, drosophila mitofusin
, mitochondrial assembly regulatory factor
, mitofusin 2
, hyperplasia suppressor
, transmembrane GTPase MFN2
, HSG protein
, hypertension related protein 1
, hypertension-related protein 1
, hypertension-related protein
, mitochondrial transmembrane GTPase FZO1A