EXOSC3 Antibody

Code CSB-PA889083LA01HU
Size US$166
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  • Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA889083LA01HU at dilution of 1:100

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) EXOSC3 Polyclonal antibody
Uniprot No.
Target Names
EXOSC3
Alternative Names
EXOSC3 antibody; RRP40 antibody; CGI-102Exosome complex component RRP40 antibody; Exosome component 3 antibody; Ribosomal RNA-processing protein 40 antibody; p10 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Exosome complex component RRP40 protein (2-275AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The EXOSC3 Antibody (Product code: CSB-PA889083LA01HU) is Non-conjugated. For EXOSC3 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA889083LB01HU EXOSC3 Antibody, HRP conjugated ELISA
FITC CSB-PA889083LC01HU EXOSC3 Antibody, FITC conjugated
Biotin CSB-PA889083LD01HU EXOSC3 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA, IHC
Recommended Dilution
Application Recommended Dilution
IHC 1:20-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Non-catalytic component of the RNA exosome complex which has 3'->5' exoribonuclease activity and participates in a multitude of cellular RNA processing and degradation events. In the nucleus, the RNA exosome complex is involved in proper maturation of stable RNA species such as rRNA, snRNA and snoRNA, in the elimination of RNA processing by-products and non-coding 'pervasive' transcripts, such as antisense RNA species and promoter-upstream transcripts (PROMPTs), and of mRNAs with processing defects, thereby limiting or excluding their export to the cytoplasm. The RNA exosome may be involved in Ig class switch recombination (CSR) and/or Ig variable region somatic hypermutation (SHM) by targeting AICDA deamination activity to transcribed dsDNA substrates. In the cytoplasm, the RNA exosome complex is involved in general mRNA turnover and specifically degrades inherently unstable mRNAs containing AU-rich elements (AREs) within their 3' untranslated regions, and in RNA surveillance pathways, preventing translation of aberrant mRNAs. It seems to be involved in degradation of histone mRNA. The catalytic inactive RNA exosome core complex of 9 subunits (Exo-9) is proposed to play a pivotal role in the binding and presentation of RNA for ribonucleolysis, and to serve as a scaffold for the association with catalytic subunits and accessory proteins or complexes. EXOSC3 as peripheral part of the Exo-9 complex stabilizes the hexameric ring of RNase PH-domain subunits through contacts with EXOSC9 and EXOSC5.
Gene References into Functions
  1. This is the first case of mitochondrial dysfunction associated with an EXOSC3 mutation, which expands the phenotypic spectrum of pontocerebellar hypoplasia type 1b. PMID: 28687512
  2. Mutations of EXOSC3/Rrp40p associated with pontocerebellar hypoplasia with progressive cerebral atrophy impact ribosomal RNA processing functions of the exosome in S. cerevisiae. PMID: 28053271
  3. EXOSC3 mutations were linked to complicated hereditary spastic paraplegia. PMID: 25149867
  4. study identified new nonsense and missense mutations in the EXOSC3 gene and showed mutations in this gene are exclusively found in pontocerebellar hypoplasia type 1 patients; there are evident genotype-phenotype correlations in EXOSC3-mediated PCH reflected in clinical outcome, age of death and pons hypoplasia PMID: 24524299
  5. The same mutation c.92G-->C, p.G31A in EXOSC3 was found in three unrelated Czech Roma patients with Pontocerebellar hypoplasia type 1 PMID: 23883322
  6. The present study indicates that EXOSC3 mutations can underlie clinical phenotype not classifiable as pontocerebellar hypoplasia type 1. PMID: 23975261
  7. We identified a homozygous mutation [c.395A > C/p.D132A] in EXOSC3 in four patients with muscle hypotonia, developmental delay, spinal anterior horn involvement, and prolonged survival, consistent with the "mild PCH1 phenotype". PMID: 23564332

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Involvement in disease
Pontocerebellar hypoplasia 1B (PCH1B)
Subcellular Location
Cytoplasm. Nucleus, nucleolus. Nucleus.
Protein Families
RRP40 family
Database Links

HGNC: 17944

OMIM: 606489

KEGG: hsa:51010

STRING: 9606.ENSP00000323046

UniGene: Hs.602571

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