Anti-EXOSC2 Antibody, Rabbit Polyclonal sinobiological 203085-T44

$143.00
In stock
SKU
203085-T44
Catalog No.SizePrice (USD)
203085-T44-5050 µL$147.96
203085-T44-100100 µL$251.64
203085-T44-200200 µL$355.32

General Information

Product nameAnti-EXOSC2 Antibody, Rabbit Polyclonal
Validated applicationsWB (FAQ Protocol), IHC-P (FAQ Protocol), IP (FAQ Protocol)
Species reactivityReacts with: Human
SpecificityHuman EXOSC2
ImmunogenE. coli-derived Human EXOSC2 fragment
PreparationProduced in rabbits immunized with E. coli-derived Human EXOSC2 fragment, and purified by antigen affinity chromatography.
SourcePolyclonal Rabbit IgG
PurificationProtein A & Antigen Affinity
FormulationPBS, pH7.0 with 0.03% Proclin300
ConjugateUnconjugated
FormLiquid
ShippingThis antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
StorageThis 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.

Synonyms: Anti-hRrp4p Antibody; Anti-p7 Antibody; Anti-RRP4 Antibody; Anti-Rrp4p Antibody; Anti-SHRF Antibody

Image / Experimental Information

Image 1 DescriptionAnti-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
Image 1 Alt TextHuman EXOSC2 Western blot (WB) 23800
Image 1 URLSource image
Image 2 DescriptionAnti-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
Image 2 Alt TextHuman EXOSC2 Immunohistochemistry(IHC) 23801
Image 2 URLSource image
Image 3 DescriptionAnti-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
Image 3 Alt TextHuman EXOSC2 Immunohistochemistry(IHC) 23802
Image 3 URLSource image
Image 4 DescriptionAnti-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
Image 4 Alt TextHuman EXOSC2 Immunoprecipitation(IP) 23803
Image 4 URLSource image

Background Information

Full Nameexosome component 2
DescriptionMissense 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
  1. 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.
  2. Morton DJ, et al. (2018) The rna exosome and rna exosome-linked disease. Rna 24 (2): 127-142.
  3. 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.

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