Code
OSM00097W
$240USD
ID Tag
Rb1589-120910-WS
Immunogen
A synthetic peptide from internal region of mouse Mitofusin2 (Mitochondrial assembly regulatory factor) conjugated to blue carrier protein was used as the antigen.
Accession
Also known
Mitofusin-2, Hypertension-related protein 1, Mitochondrial assembly regulatory factor, HSG protein, Transmembrane GTPase MFN2
Target
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.
Involvement in disease: 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.
SUBCELLULAR LOCATION: Mitochondrion outer membrane; Multi-pass membrane protein. Note: Colocalizes with BAX during apoptosis.
Tissue specificity: Ubiquitous; expressed at low level. Highly expressed in heart and kidney.
Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20C for long term storage and refrigerated at 2-8C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Chen H, et al. J. Cell Biol. 160:189-200(2003)
Limitation
For research use only
Code
OSM00097W
$240USD
Unit size
100 ul
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC, WB (confirmed by recombinant protein). A dilution of 1: 300 to 1: 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for Mitofusin-2.
Spcs X-react.
Mouse, rat, human. Not yet tested in other species.
Format
Lyophilised
Reconstitution
Reconstitute in 100 ul of sterile water. Centrifuge to remove any insoluble material.