货号：ABC42

品牌：Merck Millipore

规格：100Ug

目录价：￥4967.00

市场价格：￥4221.95

会员价格：￥3973.60

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Mitofusin-2 (Mfn2) Antibody | ABC42 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: This antibody recognizes Mitofusin-2 (Mfn2) at the coiled coil region of the cytoplasmic domain. View All» Molecular Weight: ~86 kDa observed. Mitofusion-2 (Mfn2) exists as 2 isoforms produced by alternative splicing with molecular weights of ~86 kDa and ~50 kDa. View All» Epitope: Coiled Coil Region of the Cytoplasmic Domain View All» Immunogen: KLH-conjugated linear peptide corresponding to the cytoplasmic domain of human Mitofusin-2 (Mfn2). View All» Background Information: 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. View All» Species Reactivity:

• Human

• Rat

• Mouse
  View All» Species Reactivity Note: Demonstrated to react with Human and Rat. Predicted to react with Mouse based on 100% sequence homology. View All» Control: Human heart tissue lysate View All» Quality Assurance: 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. View All» Purification Method: Affinity Purified View All» Presentation: Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: O95140 View All» Entrez Gene Number: NP_055689 View All» Gene Symbol:
  • MFN2

  • CPRP1
    View All» Alternate Names:
    • Mitofusin-2

    • Transmembrane GTPase MFN2
      View All» Usage Statement: 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. View All» Key Applications: Western Blotting View All» Entrez Gene Summary: 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]. View All» UniProt Summary: 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.10CATALYTIC 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.11TISSUE SPECIFICITY: Ubiquitous; expressed at low level. Highly expressed in heart and kidney. Ref.10 Ref.12INVOLVEMENT 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.17Defects 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. View All» Product Name: Anti-Mitofusin-2 (Mfn2) View All» Concentration: 1.0 mg/mL View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Affinity Purified View All» Host: Rabbit View All»
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