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Rabbit Anti-EXOSC3/HRP Conjugated antibody (bs-23099R-HRP)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-23099R-HRP
英文名稱1 Rabbit Anti-EXOSC3/HRP Conjugated antibody
中文名稱 辣根過氧化物酶標記的核糖體RNA合成蛋白40抗體
別    名 bA3J10.7; CGI 102; Exosome complex component RRP40; Exosome complex exonuclease RRP40; Exosome component 3; hRrp 40; hRrp40; hRrp40p; p10; PCH1B; Ribosomal RNA processing protein 40; Ribosomal RNA-processing protein 40, S. cerevisiae, homolog of; RP11-3J10.8; RRP 40; Rrp40; Rrp40p; EXOS3_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  合成與降解  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse,  (predicted: Rat, Chicken, Horse, Rabbit, Sheep, )
產(chǎn)品應用 WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 29kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human EXOSC3
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
The exosome is a multisubunit complex of 3’ to 5’ exoribonucleases. It is involved in a variety of cellular processes and is responsible for degrading unstable mRNAs that contain AU-rich elements in their untranslated 3’ region. EXOSC3 (exosome component 3), also known as p10, CGI-102, RRP40 (Ribosomal RNA-processing protein 40), Rrp40p or hRrp40p, is a component of the exosome multienzyme ribonuclease complex. Localizing to the cytoplasm and nucleolus, EXOSC3 contains a putative S1 RNA-binding domain and is capable of binding RNA. EXOSC3 is a component of the top cap of the exosome and is essential for exosome stability. In addition, EXOSC3 is required for the processing of the 7S pre-RNA to the mature 5.8S rRNA.

Function:
EXOSC3 is a component of the exosome 3'->5' exoribonuclease complex, a complex that degrades inherently unstable mRNAs containing AU-rich elements (AREs) within their 3' untranslated regions. It is required for the 3'processing of the 7S pre-RNA to the mature 5.8S rRNA. Has a 3'-5' exonuclease activity. [Swiss-Prot]

Subunit:
Component of the RNA exosome complex. Specifically part of the catalytically inactive RNA exosome core (Exo-9) complex which is believed to associate with catalytic subunits EXOSC10, and DIS3 or DIS3L in cytoplasmic- and nuclear-specific RNA exosome complex forms. Exo-9 is formed by a hexameric ring of RNase PH domain-containing subunits specifically containing the heterodimers EXOSC4-EXOSC9, EXOSC5-EXOSC8 and EXOSC6-EXOSC7, and peripheral S1 domain-containing components EXOSC1, EXOSC2 and EXOSC3 located on the top of the ring structure. Interacts with GTPBP1. Interacts with ZC3HAV1. Interacts with DDX17 only in the presence of ZC3HAV1 in an RNA-independent manner.

Subcellular Location:
Cytoplasm. Nucleus, nucleolus.

DISEASE:
Defects in EXOSC3 are the cause of pontocerebellar hypoplasia type 1B (PCH1B) [MIM:614678]. A severe autosomal recessive neurologic disorder characterized by a combination of cerebellar and spinal motor neuron degeneration beginning at birth. There is diffuse muscle weakness, progressive microcephaly, global developmental delay, and brainstem involvement.

Similarity:
Belongs to the RRP40 family.

Database links:

Entrez Gene: 51010 Human

Entrez Gene: 66362 Mouse

Entrez Gene: 313243 Rat

Omim: 606489 Human

SwissProt: Q9NQT5 Human

SwissProt: Q7TQK4 Mouse

Unigene: 602571 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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