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Mitofusin 2 Mouse mAb (bsm-60007M)  
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產(chǎn)品編號 bsm-60007M
英文名稱 Mitofusin 2 Mouse mAb
中文名稱 線粒體融合蛋白Mfn2單克隆抗體
別    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.
[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.  
研究領(lǐng)域 心血管  細胞生物  免疫學(xué)  神經(jīng)生物學(xué)  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號 G8D3
交叉反應(yīng) Human,Mouse
產(chǎn)品應(yīng)用 WB=1:500-1000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 83 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Mitofusin 2 
亞    型 IgG2a
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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



產(chǎn)品圖片
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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