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Anti-Mitofusin 2 Antibody (CAB19678)

SKU:
CAB19678
Product Type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Death
€139 - €419
Frequently bought together:

Description

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Antibody Name:Anti-Mitofusin 2 Antibody
Antibody SKU:CAB19678
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human Mitofusin 2.
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:Raji, MCF7, 293T, NIH/3T3, Mouse brain, Rat heart, Rat brain
Immunogen:A synthesized peptide derived from human Mitofusin 2.
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:9927
Uniprot:O95140
Cellular Location:
Calculated MW:80kDa
Observed MW:80KDa
Anti-Mitofusin 2 Antibody (CAB19678)Western blot - Mitofusin 2 Rabbit mAb (CAB19678)
Synonyms:CMT2A, CMT2A2, CMT2A2A, CMT2A2B, CPRP1, HMSN6A, HSG, MARF, MFN2, Mitofusin 2
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]
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Cell cycle regulation; Cytoskeletal; EC 3.6.5.-; Hydrolase; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial

Chromosomal Location of Human Ortholog: 1p36.22

Cellular Component: cytosol; integral to membrane; intrinsic to mitochondrial outer membrane; mitochondrial outer membrane; mitochondrion

Molecular Function:GTPase activity; protein binding; ubiquitin protein ligase binding

Biological Process: blood coagulation; cell cycle arrest; macroautophagy; 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

Disease: Charcot-marie-tooth Disease, Axonal, Autosomal Recessive, Type 2a2b; Charcot-marie-tooth Disease, Axonal, Type 2a2; Neuropathy, Hereditary Motor And Sensory, Type Vi

NCBI 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, Jul 2008]
UniProt Code:O95140
NCBI GenInfo Identifier:47605777
NCBI Gene ID:9927
NCBI Accession:O95140.3
UniProt Secondary Accession:O95140,O95572, Q5JXC3, Q5JXC4, Q9H131, Q9NSX8, A8K1B3
UniProt Related Accession:O95140
Molecular Weight:86kDa
NCBI Full Name:Mitofusin-2
NCBI Synonym Full Names:mitofusin 2
NCBI Official Symbol:MFN2  
NCBI Official Synonym Symbols:HSG; MARF; CMT2A; CPRP1; CMT2A2; HMSN6A; CMT2A2A; CMT2A2B  
NCBI Protein Information:mitofusin-2
UniProt Protein Name:Mitofusin-2
UniProt Synonym Protein Names:Transmembrane GTPase MFN2
Protein Family:Mitofusin
UniProt Gene Name:MFN2  

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