线粒体融合蛋白Mfn2抗体
规格：1mg/1ml
英文名： Mitofusin 2
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; T
分子量： 83kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Mitofusi
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
细胞定位：细胞浆 细胞膜
线粒体融合蛋白Mfn2抗体产品介绍：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 线粒体融合蛋白Mfn2抗体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 线粒体融合蛋白Mfn2抗体autosomal dominant form of axonal CMT associated with optic atrophy. Similarity: Belongs to the mitofusin family. Database links: Entrez Gene: 9927 Human Entrez Gene: 170731 Mouse Entrez Gene: 64476 Rat Omim: 608507 Human SwissProt: O95140 Human SwissProt: Q80U63 Mouse SwissProt: Q8R500 Rat Unigene: 376681 Human Unigene: 154312 Mouse Unigene: 437499 Mouse Unigene: 8570 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
线粒体融合蛋白Mfn2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：心血管 细胞生物 **学 神经生物学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid