Anti-Mitofusin-2 (Mfn2) (rabbit polyclonal)

Species Reactivity	Key Applications	Host	Format	Antibody Type
H, R, M	WB	Rb	Affinity Purified	Polyclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

H, R, M

Affinity Purified

Polyclonal Antibody

Description
Catalogue Number	ABC42
Description	Anti-Mitofusin-2 (Mfn2) Antibody
Alternate Names	Mitofusin-2 Transmembrane GTPase MFN2
Background Information	Mitofusin-2 (Mfn2) is a membrane protein of the mitochondria and is important in mitochondrial fusion in mammalian cells. Mfn2 activity has also been implicated in mitochondrial metabolism and the loss of Mfn2 may be the reason for certain metabolic modifications that are seen in individuals with obesity. Mfn2 has been associated with maintaining the membrane potential of the mitochondria. Mfn2 has been shown to hinder mitochondrial antiviral signaling by inhibiting production of type 1 interferons. Mfn2 is a p53-inducible target and involved in cell proliferation, apoptosis promotion and tumor suppression.

Description

Catalogue Number

ABC42

Description

Anti-Mitofusin-2 (Mfn2) Antibody

Alternate Names

Mitofusin-2
Transmembrane GTPase MFN2

Background Information

Mitofusin-2 (Mfn2) is a membrane protein of the mitochondria and is important in mitochondrial fusion in mammalian cells. Mfn2 activity has also been implicated in mitochondrial metabolism and the loss of Mfn2 may be the reason for certain metabolic modifications that are seen in individuals with obesity. Mfn2 has been associated with maintaining the membrane potential of the mitochondria. Mfn2 has been shown to hinder mitochondrial antiviral signaling by inhibiting production of type 1 interferons. Mfn2 is a p53-inducible target and involved in cell proliferation, apoptosis promotion and tumor suppression.

Product Information
Format	Affinity Purified
Control	Human heart tissue lysate
Presentation	Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Product Information

Format

Affinity Purified

Control

Human heart tissue lysate

Presentation

Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Applications
Application	Detect Mitofusin-2 (Mfn2) using this Anti-Mitofusin-2 (Mfn2) Antibody validated for use in WB.
Key Applications	Western Blotting

Applications

Application

Detect Mitofusin-2 (Mfn2) using this Anti-Mitofusin-2 (Mfn2) Antibody validated for use in WB.

Key Applications

Western Blotting

Biological Information
Immunogen	KLH-conjugated linear peptide corresponding to the cytoplasmic domain of human Mitofusin-2 (Mfn2).
Epitope	Coiled Coil Region of the Cytoplasmic Domain
Concentration	Please refer to the Certificate of Analysis for the lot-specific concentration.
Host	Rabbit
Specificity	This antibody recognizes Mitofusin-2 (Mfn2) at the coiled coil region of the cytoplasmic domain.
Species Reactivity	Human Rat Mouse
Species Reactivity Note	Demonstrated to react with Human and Rat. Predicted to react with Mouse based on 100% sequence homology.
Antibody Type	Polyclonal Antibody
Entrez Gene Number	NP_055689
Entrez Gene 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].
Gene Symbol	MFN2 CPRP1
Purification Method	Affinity Purfied
UniProt Number	O95140
UniProt Summary	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. Ref.2 Ref.9 Ref.10 CATALYTIC ACTIVITY: GTP + H2O = GDP + phosphate. SUBUNIT STRUCTURE: Forms homomultimers and heteromultimers with MFN2. Oligomerization, which is probably mediated by the coiled coil region, may play an essential role in mitochondrion fusion. SUBCELLULAR LOCATION: Mitochondrion outer membrane; Multi-pass membrane protein. Note: Colocalizes with BAX during apoptosis. Ref.9 Ref.10 Ref.11 TISSUE SPECIFICITY: Ubiquitous; expressed at low level. Highly expressed in heart and kidney. Ref.10 Ref.12 INVOLVEMENT IN 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. Ref.2 Ref.13 Ref.17 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. Sequence similarities Belongs to the mitofusin family. Sequence caution The sequence BAA34389.2 differs from that shown. Reason: Erroneous initiation. The sequence CAB70866.2 differs from that shown. Reason: Frameshift at position 581.
Molecular Weight	~86 kDa observed. Mitofusion-2 (Mfn2) exists as 2 isoforms produced by alternative splicing with molecular weights of ~86 kDa and ~50 kDa.

Biological Information

Immunogen

KLH-conjugated linear peptide corresponding to the cytoplasmic domain of human Mitofusin-2 (Mfn2).

Epitope

Coiled Coil Region of the Cytoplasmic Domain

Concentration

Please refer to the Certificate of Analysis for the lot-specific concentration.

Host

Rabbit

Specificity

This antibody recognizes Mitofusin-2 (Mfn2) at the coiled coil region of the cytoplasmic domain.

Species Reactivity

Human
Rat
Mouse

Species Reactivity Note

Demonstrated to react with Human and Rat.
Predicted to react with Mouse based on 100% sequence homology.

Antibody Type

Polyclonal Antibody

Entrez Gene Number

NP_055689

Entrez Gene 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].

Gene Symbol

MFN2
CPRP1

Purification Method

Affinity Purfied

UniProt Number

O95140

UniProt Summary

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. Ref.2 Ref.9 Ref.10

CATALYTIC ACTIVITY: GTP + H2O = GDP + phosphate.

SUBUNIT STRUCTURE: Forms homomultimers and heteromultimers with MFN2. Oligomerization, which is probably mediated by the coiled coil region, may play an essential role in mitochondrion fusion.

SUBCELLULAR LOCATION: Mitochondrion outer membrane; Multi-pass membrane protein. Note: Colocalizes with BAX during apoptosis. Ref.9 Ref.10 Ref.11

TISSUE SPECIFICITY: Ubiquitous; expressed at low level. Highly expressed in heart and kidney. Ref.10 Ref.12

INVOLVEMENT IN 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. Ref.2 Ref.13 Ref.17

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.

Sequence similarities Belongs to the mitofusin family.

Sequence caution The sequence BAA34389.2 differs from that shown. Reason: Erroneous initiation.

The sequence CAB70866.2 differs from that shown. Reason: Frameshift at position 581.

Molecular Weight

~86 kDa observed.
Mitofusion-2 (Mfn2) exists as 2 isoforms produced by alternative splicing with molecular weights of ~86 kDa and ~50 kDa.

Product Usage Statements
Quality Assurance	Evaluated by Western Blot in human heart tissue lysate. Western Blot Analysis: 0.5 µg/mL of this antibody detected Mitofusion-2 (Mfn2) in 10 µg of human heart tissue lysate.
Usage Statement	Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Product Usage Statements

Quality Assurance

Evaluated by Western Blot in human heart tissue lysate.

Western Blot Analysis: 0.5 µg/mL of this antibody detected Mitofusion-2 (Mfn2) in 10 µg of human heart tissue lysate.

Usage Statement

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Storage and Shipping Information
Storage Conditions	Stable for 1 year at 2-8°C from date of receipt.

Storage and Shipping Information

Storage Conditions

Stable for 1 year at 2-8°C from date of receipt.

Packaging Information
Material Size	100 µg

Packaging Information

Material Size

100 µg

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

办公用品

Anti-Mitofusin-2 (Mfn2) (rabbit polyclonal)

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