MFN2 Antibody
-
货号:CSB-PA013756GA01HU
-
规格:¥3,900
-
其他:
产品详情
-
Uniprot No.:O95140
-
基因名:
-
别名:MFN2; CPRP1; KIAA0214; Mitofusin-2; Transmembrane GTPase MFN2
-
宿主:Rabbit
-
反应种属:Human,Mouse,Rat
-
免疫原:Human MFN2
-
免疫原种属:Homo sapiens (Human)
-
抗体亚型:IgG
-
纯化方式:Antigen Affinity Purified
-
浓度:It differs from different batches. Please contact us to confirm it.
-
保存缓冲液:PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
-
产品提供形式:Liquid
-
应用范围:ELISA,WB,IHC
-
Protocols:
-
储存条件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
-
货期:Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
相关产品
靶点详情
-
功能:Mitochondrial outer membrane GTPase that mediates mitochondrial clustering and fusion. Mitochondria are highly dynamic organelles, and their morphology is determined by the equilibrium between mitochondrial fusion and fission events. Overexpression induces the formation of mitochondrial networks. Membrane clustering requires GTPase activity and may involve a major rearrangement of the coiled coil domains (Probable). Plays a central role in mitochondrial metabolism and may be associated with obesity and/or apoptosis processes. Plays an important role in the regulation of vascular smooth muscle cell proliferation. Involved in the clearance of damaged mitochondria via selective autophagy (mitophagy). Is required for PRKN recruitment to dysfunctional mitochondria. Involved in the control of unfolded protein response (UPR) upon ER stress including activation of apoptosis and autophagy during ER stress. Acts as an upstream regulator of EIF2AK3 and suppresses EIF2AK3 activation under basal conditions.
-
基因功能参考文献:
- The results lead to a revised understanding of Mfn 2 as single-spanning outer membrane proteins with an Nout-Cin orientation, providing functional insight into the IMS contribution to redox-regulated fusion events. PMID: 29212658
- Mfn2 protects dopaminergic neurons exposed to paraquat both in vitro and in vivo: Implications for idiopathic Parkinson's disease. PMID: 28215578
- this study provides novel insights into the tumor progression associated with MFN2 deficiency and suggests that the importance of mTORC2 inhibitor in the treatment of MFN2 downregulated cancer patients. PMID: 28176801
- Collectively, the present study demonstrated mitochondrial fission as a tumor suppression process that is regulated by the HIF/miR125a/Mfn2 pathways, acting to restrict PANC1 cell survival, energy metabolism and migration, with potential implications for novel approaches for PC therapy. PMID: 29749475
- a critical role of Mfn2 in CD4(+) T cell apoptosis in sepsis and the underlying mechanism of autophagy deficiency. PMID: 29358849
- Loss of Yap reduced SIRT1 expression and inhibited Mfn2-mediated mitophagy. Collectively, our results identified Hippo-Yap as a tumor promoter in gastric cancer that was mediated via activation of the SIRT1/Mfn2/mitophagy axis, with potential applications to gastric cancer therapy involving cancer survival and migration. PMID: 29436693
- The overexpression of Mfn2 could trigger cervical tumour apoptosis in vitro and in vivo, which was related to the mitochondrial pathway, and may provide a new treatment target for cervical carcinoma. PMID: 29587277
- Data suggest that mitofusin-2 (MFN2) may be involved in cervical cancer pathogenesis as an oncogene and might serve as a biomarker of cervical squamous cell carcinoma (SCC). PMID: 29848692
- This study detected a compound heterozygous MFN2 mutation in a patient with a severe phenotype and the co-occurrence of MFN2 and PMP22 mutations in a patient with an uncommon phenotype. PMID: 28660751
- 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. PMID: 28729638
- report of two patients with pure axonal peripheral neuropathy who are carrying novel compound heterozygous mutations in MFN2 gene PMID: 28215760
- 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 PMID: 27154191
- In this Chinese Han population a novel Charcot-Marie-Tooth disease-associated gene mutations including the MFN2 (c.497C>T) was discovered. PMID: 27862672
- Mosaicism and missense mutation in MFN2 lead to severe Charcot-Marie-Tooth disease in a daughter, with minimal clinical features in the father. PMID: 28063088
- 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 expression. PMID: 28414270
- This study identified a new mitochondria reprogramming pathway influencing breast cancer progression through SH3GL2 and MFN2. These proteins were frequently lost in breast cancer, which was traceable in the circulating exosomes. PMID: 26888829
- MFN2 expression was lower in patients with heart failure with preserved ejection fraction compared to controls. PMID: 27179829
- 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] PMID: 28132466
- 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. PMID: 26955893
- It has been shown that mitofusin-2 is modified with K6-linked polyubiquitin in a HUWE1-dependent manner. PMID: 28943312
- 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. PMID: 27553710
- 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. PMID: 28513770
- SLC25A46 is a new component in mitochondrial dynamics that serves as a regulator for MFN1/2 oligomerization. PMID: 28057766
- Presenilin 2 (PS2), mutations in which underlie familial Alzheimer's disease (FAD), promotes endoplasmic reticulum-mitochondria coupling only in the presence of mitofusin 2 (Mfn2). PMID: 27239030
- PGC-1alpha enhances Mfn2 transcription, but also leads to increased degradation of the Mfn2 protein, a key ubiquitylation target of Parkin on mitochondria. In vivo, Parkin has significant protective effects on the survival and function of nigral dopaminergic neurons in which the chronic expression of PGC-1alpha is induced PMID: 28053050
- Exome sequencing identified MFN2 SNVs in two of the individuals. Neuropathy-associated CNV outside of the PMP22 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 duplication. PMID: 26378787
- Smad2 is a key scaffold, allowing RIN1 to act as a GTP exchange factor for MFN2-GTPase activation to promote mitochondrial ATP synthesis and suppress superoxide production during mitochondrial fusion. PMID: 27184078
- our results suggest that KAP1 Ser473 phosphorylation acts through MFN2 reduction to restrict mitochondrial hyperfusion, thereby contributing to cancer cell survival under conditions of sustained metabolic stress PMID: 27364555
- Mfn2 downregulation or the exogenous expression of normal Parkin restored cytosolic Ca(2+) transients in fibroblasts from patients with PARK2 mutations, a catalytically inactive Parkinson's disease (PD)-related Parkin variant had no effect. Parkin is directly involved in regulating ER-mitochondria contacts and provide new insight into the role of the loss of Parkin function in PD development PMID: 27206984
- siRNA knockdown of mitofusin-2 (Mfn2), a protein that is involved in the tethering of endoplasmic reticulum and mitochondria, leads to increased contact between the two organelles. PMID: 27203684
- Taken together, these data suggest that the striking reduction in mitochondria in MNs expressing mutant MFN2 is not the result of impaired biogenesis, but more likely the consequence of enhanced mitophagy. PMID: 27506976
- our findings indicate miR-106a as an important factor to promote hypertrophic progress and suggest miR-106a as a new molecular target for the treatment of pathological hypertrophy. The present study also uncovered a novel relationship between miR-106a and Mfn2, with Mfn2 as a downstream signaling mediator of miR-106a. PMID: 27565029
- The results of the present study demonstrated that Resveratrol may protect bronchial epithelial cells from cigarette smoke -induced apoptosis in vitro by preventing mitochondrial dysfunction, and MFN2 may be associated with the anti-apoptotic functions of RSV in HBE cells. PMID: 28406974
- Low MFN2 expression in hepatocellular carcinoma indicated a worse overall survival. PMID: 27389277
- Low expression of MFN2 is associated with lung adenocarcinoma. PMID: 26733181
- Between 1999 and 2012, the genetic diagnosis of MFN2 mutation was made in 11 children who were treated in our department for different neurological symptoms. We found 5 different mutations in the MFN2 gene in 6 unrelated families PMID: 26686600
- We report four novel mutations and four rare missense variants of MFN2 in Charcot-Marie-Tooth disease 2A families in mainland China PMID: 26801520
- The results of this study suggested that the MFN2 gene should be considered in Polish hereditary motor-sensory neuropathiey II patients. PMID: 26581383
- These results suggest that defects in Mfn2 could cause mitochondrial dysfunction and decrease trophoblastic cells' viability PMID: 26942197
- This study demonstrated that Mfn2 gene polymorphisms were associated with essential hypertension in northern Han Chinese population, especially in male subjects PMID: 26816493
- Report exposes a novel role for Shh in regulating mitochondrial dynamics and rescue the metabolic profile of tumor cells through regulation of mitofusin 1 and 2. PMID: 26446920
- family study of early onset severe axonal Charcot-Marie-Tooth disease with dominant inheritance - SNP mutation in MFN2 PMID: 26916081
- Our findings provide new insight into the mechanism underlying Mitofusin-2 regulation and the potential role of miR-761 in tocellular carcinoma, making it a potential candidate for use in HCC therapy in the future PMID: 26845057
- HMGB1 can trigger apoptosis of T lymphocytes through mitochondrial death pathway associated with [Ca(2+)]i elevation. Mfn2 plays a pivotal role in this process, and it might be a novel therapeutic target in T cell apoptosis related disorders. PMID: 24662494
- downregulationof expression is caused by activation of resting peripheral blood T cells PMID: 26566676
- Mutations in the gene encoding MFN2 are associated with Charcot-Marie-Tooth disease type 2A and MFN2 is involved in several intracellular pathways that interact to regulate the mitochondrial network within cells. PMID: 26143526
- These findings show that homozygous mutations at p.R707W in MFN2 are a novel cause of multiple symmetrical lipomatosis. PMID: 26085578
- A deletion of exons 7 and 8 is a founder mutation in MFN2 in the UK population. PMID: 26114802
- increased expression of miR-214 observed in a Huntington disease cell model could target MFN2, altered mitochondrial morphology and deregulated cell cycle PMID: 26307536
- Mitofusin-2 over-expression leads to dysregulation of cell cycle and cell invasion in lung adenocarcinoma. PMID: 25796500
显示更多
收起更多
-
相关疾病:Charcot-Marie-Tooth disease 2A2B (CMT2A2B); Charcot-Marie-Tooth disease 2A2A (CMT2A2A); Neuropathy, hereditary motor and sensory, 6A (HMSN6A)
-
亚细胞定位:Mitochondrion outer membrane; Multi-pass membrane protein.
-
蛋白家族:TRAFAC class dynamin-like GTPase superfamily, Dynamin/Fzo/YdjA family, Mitofusin subfamily
-
组织特异性:Ubiquitous; expressed at low level. Highly expressed in heart and kidney.
-
数据库链接:
HGNC: 16877
OMIM: 601152
KEGG: hsa:9927
STRING: 9606.ENSP00000235329
UniGene: Hs.376681
Most popular with customers
-
-
YWHAB Recombinant Monoclonal Antibody
Applications: ELISA, WB, IF, FC
Species Reactivity: Human, Mouse, Rat
-
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody
Applications: ELISA, WB, IHC
Species Reactivity: Human
-
-
-
-
-