corresponding to Human Mitofusin 2. Database link: O95140
IHC-P: Human kidney tissue
This product is a recombinant rabbit monoclonal antibody.
This antibody was developed as part of a collaboration between the National Institutes of Health and the lab of Paritosh Ghosh.
We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated ‘PUR’ on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.
Produced using Abcam’s RabMAb® technology. RabMAb® technology is covered by the following U.S. Patents, No. 5,675,063 and/or 7,429,487.
功能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.
组织特异性Ubiquitous; expressed at low level. Highly expressed in heart and kidney.
疾病相关Charcot-Marie-Tooth disease 2A2 Neuropathy, hereditary motor and sensory, 6A
序列相似性Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. Mitofusin subfamily. Contains 1 dynamin-type G (guanine nucleotide-binding) domain.
翻译后修饰Phosphorylated by PINK1. Ubiquitinated by non-degradative ubiquitin by PARK2, promoting mitochondrial fusion; deubiquitination by USP30 inhibits mitochondrial fusion.
细胞定位Mitochondrion outer membrane. Colocalizes with BAX during apoptosis.
Immunofluorescence staining of HEK293 cells with purified ab124773 at a working dilution of 1/300, counter-stained with DAPI. The secondary antibody was Alexa Fluor® 488 goat anti-rabbit (ab150077), used at a dilution of 1/1000. ab7291, a mouse anti-tubulin antibody (1/1000), was used to stain tubulin along with ab150120 (Alexa Fluor® 594 goat anti-mouse, 1/1000), shown in the top right hand panel. The cells were fixed in 100% methanol and permeabilized using 0.1% Triton X 100. The negative controls are shown in bottom middle and right hand panels - for negative control 1, purified ab124773 was used at a dilution of 1/500 followed by an Alexa Fluor® 594 goat anti-mouse antibody (ab150120) at a dilution of 1/500. For negative control 2, ab7291 (mouse anti-tubulin) was used at a dilution of 1/500 followed by an Alexa Fluor® 488 goat anti-rabbit antibody (ab150077) at a dilution of 1/400.
Immunohistochemical staining of paraffin embedded human kidney with purified ab124773 at a working dilution of 1/300. The secondary antibody used is HRP goat anti-rabbit IgG H&L (ab97051) at 1/500. The sample is counter-stained with hematoxylin. Antigen retrieval was perfomed using Tris-EDTA buffer, pH 9.0. PBS was used instead of the primary antibody as the negative control, and is shown in the inset.
Western blot - Anti-Mitofusin 2 antibody [NIAR164] (ab124773)This image is courtesy of an anonymous Abreview
Anti-Mitofusin 2 antibody [NIAR164] (ab124773) at 1/1000 dilution (unpurified) + Rat primary neurons cell lysate at 20 µg
Secondary Anti-rabbit IgG HRP conjugate at 1/2000 dilution developed using the ECL technique
Cherry AD et al. Peroxisome proliferator-activated receptor ? co-activator 1-a as a critical co-activator of the murine hepatic oxidative stress response and mitochondrial biogenesis in Staphylococcus aureus sepsis. J Biol Chem289:41-52 (2014).
Read more (PubMed: 24253037) »