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Anti-Mitofusin 2 Antibody (Biotin)

 
Rabbit polyclonal antibody to Mitofusin 2 (Biotin) from FabGennix (MIT2-BIOTIN).
Overview
Name: Anti-Mitofusin 2 Antibody (Biotin)
Description: Rabbit polyclonal antibody to Mitofusin 2 (Biotin)
Applications: ELISA, IHC, IP, WB
Dilutions: DB: 1:10,000; ELISA: 1:10,000; Immunoprecipitation: 1:200; Western Blot: 1:500
Reactivity: Human, Mouse, Rat
Immunogen: Synthetic peptide taken within amino acid region 543-593 on human Mitofusin 2 protein.
Host: Rabbit
Clonality: Polyclonal
Conjugate: Biotin
Concentration: 0.6 µg/µl in antibody stabilization buffer
Storage: -20⁰C for long term storage

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. Involved in the clearance of damaged mitochondria via selective autophagy (mitophagy). Is required for PARK2 recruitment to dysfunctional mitochondria. Involved in the control of unfolded protein response (UPR) upon ER stress including activation of apoptosis and autophagy during ER stress. Acts as an upstream regulator of EIF2AK3 and suppresses EIF2AK3 activation under basal conditions.
Tissue Specificity: Ubiquitous; expressed at low level. Highly expressed in heart and kidney.
Involvement in Disease: Charcot-Marie-Tooth disease 2A2B: An axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced. CMT2A2B is a severe form with autosomal recessive inheritance.

Charcot-Marie-Tooth disease 2A2A: An axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Nerve conduction velocities are normal or slightly reduced.

Neuropathy, hereditary motor and sensory, 6A: An autosomal dominant neurologic disorder characterized by optic atrophy and peripheral sensorimotor neuropathy manifesting as axonal Charcot-Marie-Tooth disease. Charcot-Marie-Tooth disease is a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. It is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies and primary peripheral axonal neuropathies. Peripheral axonal neuropathies are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, and normal or slightly reduced nerve conduction velocities.
Sequence Similarities: Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. Mitofusin subfamily.
Post-Translational Modification: Phosphorylated by PINK1.
Cellular Location: Mitochondrion outer membrane.

Colocalizes with BAX during apoptosis.
UniProt: O95140
Synonyms: CMT2A Antibody
CMT2A2 Antibody
CPRP 1 Antibody
CPRP1 Antibody
EC 3.6.5.- Antibody
Fzo Antibody
HSG Antibody
hyperplasia suppressor gene Antibody
Hypertension related protein 1 Antibody
KIAA0214 Antibody
MARF Antibody
MFN 2 Antibody
Mfn2 Antibody
MFN2_HUMAN Antibody
Mitochondrial assembly regulatory factor Antibody
Mitofusin-2 Antibody
Mitofusin2 Antibody
Transmembrane GTPase MFN2 Antibody
Information: Target information shown above is from the UniProt Consortium.


Please Note: Anti-Mitofusin 2 Antibody (Biotin) is for research use only. It is not intended for diagnostic of therapeutic use.
Antibodies.com | MIT2-BIOTIN
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100µg £429

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