Product nameAnti-AFG3L2 antibody
See all AFG3L2 primary antibodies
DescriptionMouse polyclonal to AFG3L2
Tested applicationsSuitable for: ICC/IF, WBmore details
Species reactivityReacts with: Human
Full length protein (Human)
- AFG3L2 transfected 293T cell lysate
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.4
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab68023 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use a concentration of 5 µg/ml.|
|WB||1/500 - 1/1000. Detects a band of approximately 80 kDa (predicted molecular weight: 89 kDa).|
FunctionATP-dependent protease which is essential for axonal development.
Tissue specificityUbiquitous. Highly expressed in the cerebellar Purkinje cells.
Involvement in diseaseDefects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) [MIM:610246]. It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment.
Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5) [MIM:614487]. A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy.
Sequence similaritiesIn the N-terminal section; belongs to the AAA ATPase family.
In the C-terminal section; belongs to the peptidase M41 family.
Cellular localizationMitochondrion membrane.
- Information by UniProt
- AFG3 (ATPase family gene 3, yeast) like 2 antibody
- AFG3 ATPase family gene 3 like 2 (yeast) antibody
- AFG3 ATPase family gene 3 like 2 antibody
All lanes : Anti-AFG3L2 antibody (ab68023) at 1/500 dilution
Lane 1 : AFG3L2 transfected 293T cell lysate
Lane 2 : Non transfected 293T cell lysate
Lysates/proteins at 25 µg per lane.
All lanes : Goat Anti-Mouse IgG (H&L)-HRP Conjugate secondary
antibody at 1/2500 dilution
Developed using the ECL technique.
Predicted band size: 89 kDa
Observed band size: 80 kDa why is the actual band size different from the predicted?
ICC/IF image of ab68023 stained HepG2 cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab68023, 5µg/ml) overnight at +4°C. The secondary antibody (green) was ab96879, DyLight® 488 goat anti-mouse IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
This product has been referenced in:
- Boutoual R et al. Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPAR?-UCP2-AMPK axis. Sci Rep 8:1163 (2018). Read more (PubMed: 29348686) »
- Cesnekova J et al. Loss of Mitochondrial AAA Proteases AFG3L2 and YME1L Impairs Mitochondrial Structure and Respiratory Chain Biogenesis. Int J Mol Sci 19:N/A (2018). Read more (PubMed: 30544562) »