Name: Mitofusin-2 Polyclonal Antibody
SKU: WP13149
Price: 268 USD
Availability: InStock

Product details

Background:

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],

Specifications

Size	50μL/100μL
Target	Mitofusin-2
Reactivity	Human, Mouse, Rat
Applications	WB, IHC, IF, ELISA
MW(Calculated)
MW(Observed)	86kD
Host Species	Rabbit
Isotype	IgG
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	WB 1:500-1:2000; IHC: 1:100-300; ELISA 1:20000; IF 1:100-300; Not yet tested in other applications. / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Purification	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration	1 mg/ml
Clonality	Polyclonal
Clone Number
Immunogen	The antiserum was produced against synthesized peptide derived from human Mfn2. AA range:354-403
Specificity	Mfn2 Polyclonal Antibody detects endogenous levels of Mfn2 protein.
Gene Name	MFN2
Protein Name	Mitofusin-2
Other Name	MFN2; / CPRP1; / KIAA0214; / Mitofusin-2; / Transmembrane GTPase MFN2
Species	Human
Gene ID	9927
Uniprot	O95140,
Species.1	Mouse
Gene ID.1	170731
Uniprot.1	Q80U63,
Species.2	Rat
Gene ID.2
Uniprot.2	Q8R500
Cellular Localization	Mitochondrion outer membrane; Multi-pass membrane protein . Colocalizes with BAX during apoptosis. .
Tissue Expression	Ubiquitous; expressed at low level. Highly expressed in heart and kidney.
Signaling_pathway	Cellular Processes >> Transport and catabolism >> Mitophagy - animal;Organismal Systems >> Immune system >> NOD-like receptor signaling pathway;Human Diseases >> Neurodegenerative disease >> Parkinson disease;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases
Research Areas	>>Mitophagy - animal; / >>NOD-like receptor signaling pathway; / >>Parkinson disease; / >>Pathways of neurodegeneration - multiple diseases
Function	Catalytic activity:GTP + H(2)O = GDP + phosphate.,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.,Disease: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.,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.,similarity:Belongs to the mitofusin family.,subcellular location:Colocalizes with BAX during apoptosis.,subunit:Forms homomultimers and heteromultimers with MFN2. Oligomerization, which is probably mediated by the coiled coil region, may play an essential role in mitochondrion fusion.,tissue specificity:Ubiquitous; expressed at low level. Highly expressed in heart and kidney.,