Rabbit Mitofusin 2 Polyclonal Antibody | anti-MFN2 antibody
Mitofusin 2 Antibody BIOTIN-Conjugated
ELISA: 1:10,000
Immunohistochemistry: 1:100
Immunoprecipitation: 1:200
Western Blot: 1:500
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
MFN2: 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. Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 2A2 (CMT2A2). 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); 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. Belongs to the mitofusin family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.6.5.-; Cell cycle regulation; Hydrolase; Cytoskeletal; Membrane protein, multi-pass; Mitochondrial; Membrane protein, integral
Chromosomal Location of Human Ortholog: 1p36.22
Cellular Component: cytosol; integral to membrane; intrinsic to mitochondrial outer membrane; microtubule cytoskeleton; mitochondrial outer membrane; mitochondrion
Molecular Function: GTP binding; GTPase activity; protein binding; ubiquitin protein ligase binding
Biological Process: apoptosis; blastocyst formation; blood coagulation; camera-type eye morphogenesis; cell cycle arrest; macroautophagy; metabolic process; mitochondrial fusion; mitochondrial membrane organization and biogenesis; mitochondrion localization; negative regulation of Ras protein signal transduction; negative regulation of smooth muscle cell proliferation; protein targeting to mitochondrion; response to unfolded protein
Disease: Charcot-marie-tooth Disease, Axonal, Type 2a2; Neuropathy, Hereditary Motor And Sensory, Type Vi
Research Articles on MFN2
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Product Notes
The MFN2 mfn2 (Catalog #AAA542101) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Mitofusin 2 Antibody BIOTIN-Conjugated reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Mitofusin 2 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunohistochemistry (IHC), Immunoprecipitation (IP), Western Blot (WB). Dot Blot: 1:10,000 ELISA: 1:10,000 Immunohistochemistry: 1:100 Immunoprecipitation: 1:200 Western Blot: 1:500. Researchers should empirically determine the suitability of the MFN2 mfn2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Mitofusin 2, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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