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Human MFN2 Protein expressed in HEK-293 Cells - ABIN2726078
Wang, Zhang, Li, Tang, Siedlak, Fujioka, Liu, Su, Pi, Wang: MFN2 couples glutamate excitotoxicity and mitochondrial dysfunction in motor neurons. in The Journal of biological chemistry 2015
Enhancing the profusion gene mitofusin/marf is beneficial in an in vivo model of TDP-43 (show TARDBP Proteins) proteinopathies, serving as a potential therapeutic target.
activation of endoplasmic reticulum stress by defective mitochondria is neurotoxic in pink1 (show PINK1 Proteins) and parkin (show PARK2 Proteins) flies and that the reduction of this signalling is neuroprotective, independently of defective mitochondria.
Clu (show CLU Proteins) is upstream of and binds to VCP (show vcp Proteins) in vivo and promotes VCP (show vcp Proteins)-dependent Marf degradation in vitro Marf accumulates in whole muscle lysates of clu (show CLU Proteins)-deficient flies and is destabilized upon Clu (show CLU Proteins) overexpression. Thus, Clu (show CLU Proteins) is essential for mitochondrial homeostasis and functions in concert with Parkin (show PARK2 Proteins) and VCP (show vcp Proteins) for Marf degradation to promote damaged mitochondrial clearance.
lack of ChChd3 (show CHCHD3 Proteins) leads to inactivation of Hippo activity under normal development, which is also dependent on the transcriptional coactivator Yorkie (Yki (show YAP1 Proteins)). Furthermore, loss of ChChd3 (show CHCHD3 Proteins) induces oxidative stress and activates the JNK (show MAPK8 Proteins) pathway. In addition, depletion of other mitochondrial fusion components, Opa1 (show OPA1 Proteins) 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 Proteins) 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 Proteins) control mitochondrial and cardiac function in Drosophila.
The research findings indicate that the inhibition of microRNA-214 promotes the epithelial mesenchymal transition process and contributes to bladder wall fibrosis by up-regulating Mitofusin 2, thus leading to the occurrence of interstitial cystitis in postmenopausal women.
report of two patients with pure axonal peripheral neuropathy who are carrying novel compound heterozygous mutations in MFN2 gene
The heterozygous mutation c.2251C>T was identified in exon 19 of the MFN2 gene, presumably leading to the truncation of the MFN2 protein (p.Gln751Ter). The mutation co-segregated completely with the disease within the family
In this Chinese Han population a novel Charcot-Marie-Tooth disease-associated gene mutations including the MFN2 (c.497C>T) was discovered.
Mosaicism and missense mutation in MFN2 lead to severe Charcot-Marie-Tooth disease in a daughter, with minimal clinical features in the father.
These findings suggest that specific MFN2 mutations cause tissue-selective mitochondrial dysfunction with increased adipocyte proliferation and survival and confirm a novel form of excess adiposity with paradoxical suppression of leptin (show LEP Proteins) expression.
This study identified a new mitochondria reprogramming pathway influencing breast cancer progression through SH3GL2 (show SH3G2 Proteins) and MFN2. These proteins were frequently lost in breast cancer, which was traceable in the circulating exosomes.
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.
BAT (show BAAT Proteins) (brown adipose tissue) adaptation to obesity is regulated by Mfn2 and with BAT (show BAAT Proteins)-Mfn2 absent, BAT (show BAAT Proteins) contribution to prevention of insulin (show INS Proteins) resistance is independent and inversely correlated to whole-body cold-stimulated thermogenesis.
Mfn2 stands as a bona fide endoplasmic-mitochondria tether whose ablation decreases interorganellar juxtaposition and communication.
Despite apparent mitochondrial dysfunction, hearts deficient in both Mfn1 (show MFN1 Proteins) and Mfn2 are protected against acute myocardial infarction due to impaired mitochondria/sarcoplasmic reticulum tethering.
Presenilin 2 (PS2 (show PSEN2 Proteins)), mutations in which underlie familial Alzheimer's disease (FAD), 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 Proteins)-195. Therefore, upregulation of mfn2 expression by decreasing the level of miR (show MLXIP Proteins)-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 Proteins) restored cytosolic Ca(2 (show CA2 Proteins)+) transients in fibroblasts from patients with PARK2 (show PARK2 Proteins) mutations, a catalytically inactive Parkinson's disease (PD)-related Parkin (show PARK2 Proteins) variant had no effect. Parkin (show PARK2 Proteins) is directly involved in regulating ER-mitochondria contacts and provide new insight into the role of the loss of Parkin (show PARK2 Proteins) 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 Proteins)+, PPAR-b (show PPARD Proteins) took an important role in neuronal differentiation induced by flavonoid compound 4a
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 Proteins).
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