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DIS3L2 Rabbit pAb (bs-9053R)  
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產(chǎn)品編號 bs-9053R
英文名稱 DIS3L2 Rabbit pAb
中文名稱 有絲分裂控制蛋白樣DIS3L2抗體
別    名 DIS3 L2; DIS3 mitotic control homolog(S. cerevisiae) like 2; DIS3 mitotic control homolog like 2; FLJ36974; MGC42174; DI3L2_HUMAN.  
研究領(lǐng)域 心血管  細(xì)胞生物  免疫學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse (predicted: Human,Rat,Pig,Sheep,Cow,Dog)
產(chǎn)品應(yīng)用 Flow-Cyt=2ug/Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 99 kDa
檢測分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DIS3L2: 1-100/885 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 The exosome is a multisubunit complex composed of several highly conserved subunits, some of which are 3’ to 5’ exoribonucleases. The complex is involved in a variety of cellular processes and is responsible for degrading unstable mRNAs that contain AU-rich (ARE) elements in their untranslated 3’ region. DIS3L2 (DIS3-like exonuclease 2) is an 885 amino acid protein that is thought to function as an exonuclease and may be required for the 3’ processing of pre-mRNA into mature mRNA. Defects or chromosomal translocations involving the gene encoding DIS3L2 may be associated with Marfanoid habitus, a genetic disorder characterized by abnormalities in the skeleton, eyes and cardiovascular system. DIS3L2 is expressed as five isoforms due to alternative splicing events.

Function:
Ribonuclease that plays a critical role in RNA metabolism. It is essential for correct mitosis, and negatively regulates cell proliferation.

Subcellular Location:
Cytoplasm.

DISEASE:
Defects in DIS3L2 are the cause of Perlman syndrome (PRLMNS) [MIM:267000]. An autosomal recessive congenital overgrowth syndrome. Affected children are large at birth, are hypotonic, and show organomegaly, characteristic facial dysmorphisms (inverted V-shaped upper lip, prominent forehead, deep-set eyes, broad and flat nasal bridge, and low-set ears), renal anomalies (nephromegaly and hydronephrosis), frequent neurodevelopmental delay, and high neonatal mortality. Perlman syndrome is associated with a high risk of Wilms tumor. Histologic examination of the kidneys in affected children shows frequent nephroblastomatosis, which is a precursor lesion for Wilms tumor.

Similarity:
Belongs to the RNR ribonuclease family.

SWISS:
Q8IYB7

Gene ID:
129563

Database links:

Entrez Gene: 129563 Human

Entrez Gene: 208718 Mouse

Entrez Gene: 367307 Rat

Omim: 614184 Human

SwissProt: Q8IYB7 Human

SwissProt: Q8CI75 Mouse

Unigene: 732236 Human

Unigene: 389152 Mouse

Unigene: 2940 Rat



產(chǎn)品圖片
Blank control:Mouse kidney. Primary Antibody (green line): Rabbit Anti-DIS3L2 antibody (bs-9053R) Dilution: 2μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody : Goat anti-rabbit IgG-AF488 Dilution: 1μg /test. Protocol The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 0.1% PBST for 20 min at room temperature. The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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