WH0009927M3

Sigma-Aldrich

Monoclonal Anti-MFN2 antibody produced in mouse

clone 4H8, purified immunoglobulin, buffered aqueous solution

Synonim(y):
Anti-MARF, Anti-mitofusin 2, Anti-HSG, Anti-KIAA0214, Anti-CMT2A2, Anti-CMT2A, Anti-CPRP1
Numer MDL:
NACRES:
NA.41

Poziom jakości

100

pochodzenie biologiczne

mouse

forma przeciwciała

purified immunoglobulin

antibody product type

primary antibodies

klon

4H8, monoclonal

formularz

buffered aqueous solution

species reactivity

human, rat, mouse

zastosowania

immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL

izotyp

IgG2aκ

białko sprzężone

unconjugated

GenBank® accession no.

numer dostępu UniProt

wysyłka w ciągu

dry ice

temp. przechowywania

−20°C

Gene Information

human ... MFN2(9927)

Opis ogólny

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)

Immunogen

MFN2 (NP_055689, 661 a.a. ~ 757 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
FKRQFVEHASEKLQLVISYTGSNCSHQVQQELSGTFAHLCQQVDVTRENLEQEIAAMNKKIEVLDSLQSKAKLLRNKAGWLDSELNMFTHQYLQPSR

Działania biochem./fizjol.

Mitofusin 2 (MFN2) plays an essential role in mitochondrial metabolism via maintenance and operation of the mitochondrial network architecture through fusion of mitochondria. Decreased expression of MFN2 leads to metabolic abnormalities, which further increases the risk of developing obesity and type 2 diabetes. Mutation of the gene results in Charcot–Marie–Tooth neuropathy type 2A (CMT2A). Mfn2 controls various cellular function including cell proliferation, oxidative metabolism, autophagy, and mitochondrial antiviral signaling protein. MFN2 integrates mitochondria and endoplasmic reticulum function, which is required for regulation of insulin signaling and glucose homeostasis in vivo. Repressed expression of MFN2 in placenta is associated with pathogenesis of preeclampsia (PE). MFN2 functions as a biomarker and therapeutic target for PE and cardiovascular diseases, such as hypertension.

Postać fizyczna

Solution in phosphate buffered saline, pH 7.4

Informacje prawne

GenBank is a registered trademark of United States Department of Health and Human Services

Oświadczenie o zrzeczeniu się odpowiedzialności

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_class_code

12 - Non Combustible Liquids

Temperatura zapłonu °F

Not applicable

Temperatura zapłonu °C

Not applicable

Środki ochrony indywidualnej

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

Certyfikat analizy

Świadectwo pochodzenia

Kevin R Murphy et al.
Frontiers in physiology, 10, 1277-1277 (2019-10-23)
Aging in humans is associated with a 10-40-fold greater incidence of sudden cardiac death from malignant tachyarrhythmia. We have reported that thiol oxidation of ryanodine receptors (RyR2s) by mitochondria-derived reactive oxygen species (mito-ROS) contributes to defective Ca2+ homeostasis in cardiomyocytes...
Du Fang et al.
Scientific reports, 6, 31462-31462 (2016-08-19)
Mitochondria are essential dynamic organelles for energy production. Mitochondria dynamically change their shapes tightly coupled to fission and fusion. Imbalance of fission and fusion can cause deficits in mitochondrial respiration, morphology and motility. Mfn2 (mitofusin 2), a mitochondrial membrane protein...
Daniel Bach et al.
The Journal of biological chemistry, 278(19), 17190-17197 (2003-02-25)
In many cells and specially in muscle, mitochondria form elongated filaments or a branched reticulum. We show that Mfn2 (mitofusin 2), a mitochondrial membrane protein that participates in mitochondrial fusion in mammalian cells, is induced during myogenesis and contributes to...
David Sebastián et al.
Proceedings of the National Academy of Sciences of the United States of America, 109(14), 5523-5528 (2012-03-20)
Mitochondria are dynamic organelles that play a key role in energy conversion. Optimal mitochondrial function is ensured by a quality-control system tightly coupled to fusion and fission. In this connection, mitofusin 2 (Mfn2) participates in mitochondrial fusion and undergoes repression...
Shireen A Sarraf et al.
Nature, 496(7445), 372-376 (2013-03-19)
The PARKIN ubiquitin ligase (also known as PARK2) and its regulatory kinase PINK1 (also known as PARK6), often mutated in familial early-onset Parkinson's disease, have central roles in mitochondrial homeostasis and mitophagy. Whereas PARKIN is recruited to the mitochondrial outer...

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