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品种反应性 主要应用 宿主 格式 抗体类型 M, H, R WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 ABC42 描述 Anti-Mitofusin-2 (Mfn2) Antibody Alternate Names - Transmembrane GTPase MFN2
- Mitofusin-2
背景信息 Mitofusin-2 (Mfn2) is a membrane protein of the mitochondria and is important in mitochondrial fusion in mammalian cells. Mfn2 activity has also been implicated in mitochondrial metabolism and the loss of Mfn2 may be the reason for certain metabolic modifications that are seen in individuals with obesity. Mfn2 has been associated with maintaining the membrane potential of the mitochondria. Mfn2 has been shown to hinder mitochondrial antiviral signaling by inhibiting production of type 1 interferons. Mfn2 is a p53-inducible target and involved in cell proliferation, apoptosis promotion and tumor suppression. 产品信息 格式 Affinity Purified 控制 - Human heart tissue lysate
演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Detect Mitofusin-2 (Mfn2) using this Anti-Mitofusin-2 (Mfn2) Antibody validated for use in WB. 主要应用 - Western Blotting
生物信息 免疫原品种 KLH-conjugated linear peptide corresponding to the cytoplasmic domain of human Mitofusin-2 (Mfn2). 表位 Coiled Coil Region of the Cytoplasmic Domain 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 特异性 This antibody recognizes Mitofusin-2 (Mfn2) at the coiled coil region of the cytoplasmic domain. 品种反应性 MouseHumanRat Species Reactivity Note Demonstrated to react with Human and Rat.
Predicted to react with Mouse based on 100% sequence homology.抗体类型 Polyclonal Antibody Entrez基因编号 - NP_055689
Entrez基因汇总 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]. 基因符号 - CPRP1
- MFN2
纯化方法 Affinity Purified UniProt编号 - O95140
UniProt汇总 FUNCTION: Essential transmembrane GTPase, which mediates mitochondrial fusion. Fusion of mitochondria occurs in many cell types and constitutes an important step in mitochondria morphology, which is balanced between fusion and fission. MFN2 acts independently of the cytoskeleton. It therefore plays a central role in mitochondrial metabolism and may be associated with obesity and/or apoptosis processes. Overexpression induces the formation of mitochondrial networks. Plays an important role in the regulation of vascular smooth muscle cell proliferation. Ref.2 Ref.9 Ref.10
CATALYTIC ACTIVITY: GTP + H2O = GDP + phosphate.
SUBUNIT STRUCTURE: Forms homomultimers and heteromultimers with MFN2. Oligomerization, which is probably mediated by the coiled coil region, may play an essential role in mitochondrion fusion.
SUBCELLULAR LOCATION: Mitochondrion outer membrane; Multi-pass membrane protein. Note: Colocalizes with BAX during apoptosis. Ref.9 Ref.10 Ref.11
TISSUE SPECIFICITY: Ubiquitous; expressed at low level. Highly expressed in heart and kidney. Ref.10 Ref.12
INVOLVEMENT IN 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. Ref.2 Ref.13 Ref.17
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.
Sequence similarities Belongs to the mitofusin family.
Sequence caution The sequence BAA34389.2 differs from that shown. Reason: Erroneous initiation.
The sequence CAB70866.2 differs from that shown. Reason: Frameshift at position 581.产品使用声明 质量保证 Evaluated by Western Blot in human heart tissue lysate.
Western Blot Analysis: 0.5 µg/mL of this antibody detected Mitofusion-2 (Mfn2) in 10 µg of human heart tissue lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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