WB (FAQ Protocol), IHC-P (FAQ Protocol), IP (FAQ Protocol)
Species reactivity
Reacts with: Human
Specificity
Human EXOSC2
Immunogen
E. coli-derived Human EXOSC2 fragment
Preparation
Produced in rabbits immunized with E. coli-derived Human EXOSC2 fragment, and purified by antigen affinity chromatography.
Source
Polyclonal Rabbit IgG
Purification
Protein A & Antigen Affinity
Formulation
PBS, pH7.0 with 0.03% Proclin300
Conjugate
Unconjugated
Form
Liquid
Shipping
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Storage
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Avoid repeated freeze-thaw cycles.
Anti-EXOSC2 rabbit polyclonal antibody at 1:500 dilution Lane A: 293T Whole Cell Lysate Lane B: Hela Whole Cell Lysate Lane C: Jurkat Whole Cell Lysate Lysates/proteins at 30 μg 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:33 kDa Observed band size:33 kDa
Anti-EXOSC2 rabbit polyclonal antibody at 1:500 dilution Lane A: 293T Whole Cell Lysate Lane B: Hela Whole Cell Lysate Lane C: Jurkat Whole Cell Lysate Lysates/proteins at 30 μg 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:33 kDa Observed band size:33 kDa
Anti-EXOSC2 rabbit polyclonal antibody at 1:500 dilution Lane A: 293T Whole Cell Lysate Lane B: Hela Whole Cell Lysate Lane C: Jurkat Whole Cell Lysate Lysates/proteins at 30 μg 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:33 kDa Observed band size:33 kDa
Anti-EXOSC2 rabbit polyclonal antibody at 1:500 dilution Lane A: 293T Whole Cell Lysate Lane B: Hela Whole Cell Lysate Lane C: Jurkat Whole Cell Lysate Lysates/proteins at 30 μg 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:33 kDa Observed band size:33 kDa
Missense mutations in the RNA exosome component 2 (EXOSC2), also known as ribosomal RNA-processing protein 4 (RRP4), were recently identified in two unrelated families with a novel syndrome known as Short stature, Hearing loss, Retinitis pigmentosa, and distinctive Facies (SHRF). EXOSC2 encodes the 'ribosomal RNA-processing protein 4' (RRP4)-one of the core components of the RNA exosome. The RNA exosome is a multiprotein complex that plays key roles in RNA processing and degradation. Mutations in the RNA exosome gene EXOSC2 cause a distinct syndrome with various tissue-specific phenotypes including retinitis pigmentosa, hearing loss, and mild intellectual disability. Retinitis pigmentosa in combination with hearing loss can be a feature of different Mendelian disorders.
References
Yang X, et al. (2020) Genetic and genomic studies of pathogenic exosc2 mutations in the newly described disease shrf implicate the autophagy pathway in disease pathogenesis. Hum Mol Genet 29 (4): 541-553.
Morton DJ, et al. (2018) The rna exosome and rna exosome-linked disease. Rna 24 (2): 127-142.
Di Donato N, et al. (2016) Mutations in exosc2 are associated with a novel syndrome characterised by retinitis pigmentosa, progressive hearing loss, premature ageing, short stature, mild intellectual disability and distinctive gestalt. J Med Genet 53 (6): 419-425.