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MouseAnti-Mitofusin 2 Mouse mAb  antibody (bsm-60007M)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  
50ul/1180.00元
100ul/1980.00元
大包装/询价

产品编号 bsm-60007M
英文名称 Mitofusin 2 Mouse mAb
中文名称 线粒体融合蛋白Mfn2单克隆抗体
别    名 CMT2A; CMT2A2; MARF; CPRP 1; CPRP1; Fzo; HSG; hyperplasia suppressor gene; Hypertension related protein 1; MFN 2; Mfn2; MFN2_HUMAN; Mitochondrial assembly regulatory factor; Mitofusin-2; Mitofusin2; Transmembrane GTPase MFN2.  
Specific References  (1)     |     bsm-60007M has been referenced in 1 publications.
111 [IF=5.572] Miao Song. et al. Mitophagy alleviates AIF-mediated spleen apoptosis induced by AlCl3 through Parkin stabilization in mice. FOOD CHEM TOXICOL. 2023 Jun;176:113762  WB ;  Mouse.  222
研究领域 心血管  细胞生物  免疫学  神经生物学  
抗体来源 Mouse
克隆类型 Monoclonal
克 隆 号 G8D3
交叉反应 Human, Mouse, 
产品应用 WB=1:500-2000 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 83 kDa
检测分子量
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Mitofusin 2 
亚    型 IgG2a
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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. [provided by RefSeq, Jul 2008].

Subunit:
Forms homomultimers and heteromultimers with MFN1.

Subcellular Location:
Mitochondrion outer membrane; Multi-pass membrane protein.

Tissue Specificity:
Ubiquitous; expressed at low level. Highly expressed in heart and kidney.

DISEASE:
Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 2A2 (CMT2A2) [MIM:609260]. CMT2A2 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 6 (CMT6) [MIM:601152]; also referred to as autosomal dominant hereditary motor and sensory neuropathy VI (HMSN6). CMT6 is an autosomal dominant form of axonal CMT associated with optic atrophy.

Similarity:
Belongs to the mitofusin family.

SWISS:
O95140

Gene ID:
9927

Database links:

Entrez Gene: 9927 Human

Entrez Gene: 170731 Mouse

Entrez Gene: 64476 Rat

SwissProt: O95140 Human

SwissProt: Q80U63 Mouse

SwissProt: Q8R500 Rat

Unigene: 376681 Human



产品图片
Sample: Lane 1: Mouse NIH/3T3 cell lysates Lane 2: Human HeLa cell lysates Lane 3: Human HepG2 cell lysates Primary: Anti-Mitofusin 2 (bsm-60007M) at 1/1000 dilution Secondary: IRDye800CW Goat Anti- Mouse IgG at 1/20000 dilution Predicted band size: 83 kDa Observed band size: 76 kDa
Blocking buffer: 5% NFDM/TBST Primary ab dilution: 1:1000 Primary ab incubation condition: 2 hours at room temperature Lysate: HepG2, Neuro-2a, EL4.IL-2 Protein loading quantity: 20 μg Exposure time: 60 S Predicted MW: 86 kDa Observed MW: 86 kDa
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