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货号： bs-2988R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-2988R-AF350
- 英文名称
- Anti-Mitofusin 2/AF350
- 中文名称
- AF350标记的线粒体融合蛋白Mfn2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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 心血管 细胞生物 免疫学 神经生物学
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 83kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Mitofusin 2
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
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.
Database links:Entrez Gene: 9927Human
Entrez Gene: 170731Mouse
Entrez Gene: 64476Rat
Omim: 608507Human
SwissProt: O95140Human
SwissProt: Q80U63Mouse
SwissProt: Q8R500Rat
Unigene: 376681Human
Unigene: 154312Mouse
Unigene: 437499Mouse
Unigene: 8570Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.