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ABC42 Anti-Mitofusin-2 (Mfn2) Antibody

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ABC42
100 µg  
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      Tabella delle specifiche principali

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, R, MWBRbAffinity PurifiedPolyclonal Antibody
      Description
      Catalogue NumberABC42
      DescriptionAnti-Mitofusin-2 (Mfn2) Antibody
      Alternate Names
      • Mitofusin-2
      • Transmembrane GTPase MFN2
      Background InformationMitofusin-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.
      References
      Product Information
      FormatAffinity Purified
      Control
      • Human heart tissue lysate
      PresentationPurified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationDetect Mitofusin-2 (Mfn2) using this Anti-Mitofusin-2 (Mfn2) Antibody validated for use in WB.
      Key Applications
      • Western Blotting
      Biological Information
      ImmunogenKLH-conjugated linear peptide corresponding to the cytoplasmic domain of human Mitofusin-2 (Mfn2).
      EpitopeCoiled Coil Region of the Cytoplasmic Domain
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostRabbit
      SpecificityThis antibody recognizes Mitofusin-2 (Mfn2) at the coiled coil region of the cytoplasmic domain.
      Species Reactivity
      • Human
      • Rat
      • Mouse
      Species Reactivity NoteDemonstrated to react with Human and Rat.
      Predicted to react with Mouse based on 100% sequence homology.
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis 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].
      Gene Symbol
      • MFN2
      • CPRP1
      Purification MethodAffinity Purfied
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      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.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated 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.
      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.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Numero di catalogo GTIN
      ABC42 04053252558276

      Documentation

      Anti-Mitofusin-2 (Mfn2) Antibody MSDS

      Titolo

      Scheda di sicurezza (MSDS) 

      Anti-Mitofusin-2 (Mfn2) Antibody Certificati d'Analisi

      TitoloNumero di lotto
      Anti-Mitofusin-2 (Mfn2) - 2382639 2382639
      Anti-Mitofusin-2 (Mfn2) - 2433629 2433629
      Anti-Mitofusin-2 (Mfn2) - 2053957 2053957
      Anti-Mitofusin-2 (Mfn2) - 2301290 2301290
      Anti-Mitofusin-2 (Mfn2) - 3182684 3182684
      Anti-Mitofusin-2 (Mfn2) - 3439721 3439721
      Anti-Mitofusin-2 (Mfn2) - NRG1890871 NRG1890871
      Anti-Mitofusin-2 (Mfn2) -2592772 2592772
      Anti-Mitofusin-2 (Mfn2) -2705134 2705134
      Anti-Mitofusin-2 (Mfn2) -2818695 2818695

      Riferimenti bibliografici

      Panoramica dei riferimenti bibliograficiCodice d'identificazione nel Pub Med
      Differentiation state-specific mitochondrial dynamic regulatory networks are revealed by global transcriptional analysis of the developing chicken lens.
      Chauss, D; Basu, S; Rajakaruna, S; Ma, Z; Gau, V; Anastas, S; Brennan, LA; Hejtmancik, JF; Menko, AS; Kantorow, M
      G3 (Bethesda, Md.)  4  1515-27  2014

      Mostra il sommario
      24928582 24928582

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      Categorie

      Life Science Research > Antibodies and Assays > Primary Antibodies