Rabbit anti-Human AFG3L2 Polyclonal Antibody | anti-AFG3L2 antibody

Anti-AFG3L2 Antibody, Rabbit Polyclonal

Cat. Num. AAA8108970

• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry, Immunocytochemistry, Immunofluorescence, Immunoprecipitation
• Purity: Protein A & Antigen Affinity
• Synonyms: AFG3L2; Polyclonal Antibody; Anti-AFG3L2 Antibody; Rabbit Polyclonal; AFG3L2 Antibody; Rabbit PAb; Antigen Affinity Purified; AFG3-like AAA ATPase 2; Anti-SCA28 Antibody; Anti-SPAX5 Antibody; anti-AFG3L2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human AFG3L2
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; PBS, pH7.0 with 0.03% Proclin300

• Applicable Applications for anti-AFG3L2 antibody: Western Blot (WB), Immunohistochemistry-Paraffin (IHC-P), Immunocytochemistry (ICC), Immunofluorescence (IF), Immunoprecipitation (IP)
• Application Notes: WB: 1:500-1:2000; IHC-P: 1:500-1:2000; ICC/IF: 1:100-1:500; IP: 5-10uL/mg of lysate
• Immunogen: E Coli-derived Human AFG3L2 fragment
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with E Coli-derived Human AFG3L2 fragment, and purified by antigen affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

(Anti-AFG3L2 rabbit polyclonal antibody at 1:500 dilution Lane A: Hela Whole Cell Lysate Lane B: MDA-MB453 Whole Cell Lysate Lane C: MCF-7 Whole Cell Lysate Lane D: Jurkat Whole Cell Lysate Lysates/proteins at 30 ug per lane. Secondary Goat Anti-Rabbit IgG (H+L)/HRP at 1/10000 dilution. Developed using the ECL technique. Performed under reducing conditions. Predicted band size:89 kDa Observed band size:95 kDa (We are unsure as to the identity of these extra bands.))

Immunohistochemistry (IHC)

(Immunochemical staining of human AFG3L2 in human kidney with rabbit polyclonal antibody at 1:1000 dilution, formalin-fixed paraffin embedded sections.)

Immunohistochemistry (IHC)

(Immunochemical staining of human AFG3L2 in human duodenum with rabbit polyclonal antibody at 1:1000 dilution, formalin-fixed paraffin embedded sections.)

Immunofluorescence (IF)

(Immunofluorescence staining of AFG3L2 in U251MG cells. Cells were fixed with 4% PFA, permeabilzed with 0.1% Triton X-100 in PBS,blocked with 10% serum, and incubated with rabbit anti-Human AFG3L2 polyclonal antibody (dilution ratio 1:200) at 4 degree C overnight. Then cells were stained with the Alexa Fluor488-conjugated Goat Anti-rabbit IgG secondary antibody (green) and counterstained with DAPI (blue).Positive staining was localized to Cytoplasm.)

Immunoprecipitation (IP)

(AFG3L2 was immunoprecipitated using: Lane A:0.5 mg HeLa Whole Cell Lysate 4 uL anti-AFG3L2 rabbit polyclonal antibody and 60 ug of Immunomagnetic beads Protein A/G. Primary antibody: Anti-AFG3L2 rabbit polyclonal antibody,at 1:100 dilution Secondary antibody: Goat Anti-Rabbit IgG (H+L)/HRP at 1/10000 dilution Developed using the ECL technique. Performed under reducing conditions. Predicted band size: 89 kDa Observed band size :95 kDa)

Related Product Information for anti-AFG3L2 antibody

Human AFG3L2 is a compartmental AAA+ protease that performs ATP-fueled degradation at the matrix face of the inner mitochondrial membrane. Paraplegin and AFG3L2 are ubiquitous nuclear-encoded mitochondrial proteins that form hetero-oligomeric paraplegin-AFG3L2 and homo-oligomeric AFG3L2 complexes in the inner mitochondrial membrane, named m-AAA proteases. These complexes are in charge of quality control of misfolded proteins and participate in the regulation of OPA1 proteolytic cleavage, required for mitochondrial fusion. Mutations in AFG3L2 cause spinocerebellar ataxia type 28 and a complex neurodegenerative syndrome of childhood. Identifying how AFG3L2 selects substrates from the diverse complement of matrix-localized proteins is essential for understanding mitochondrial protein biogenesis and quality control.

References

Maltecca F, et al. (2012) Respiratory dysfunction by afg3l2 deficiency causes decreased mitochondrial calcium uptake via organellar network fragmentation. Hum Mol Genet 21 (17): 3858-3870.Maltecca F, et al. (2009) Haploinsufficiency of afg3l2, the gene responsible for spinocerebellar ataxia type 28, causes mitochondria-mediated purkinje cell dark degeneration. J Neurosci 29 (29): 9244-9254.Ding B, et al. (2018) Dissecting substrate specificities of the mitochondrial afg3l2 protease. Biochemistry 57 (28): 4225-4235.