Anti-DIS3L2抗体The exosome is a multisubunit complex composed of several highly conserved subunits, some of which are 3’ to 5’

产品编号 yb-9053R
英文名称 Anti-DIS3L2抗体
中文名称 有丝分裂控制蛋白样DIS3L2抗体
别    名 DIS3 L2; DIS3 mitotic control homolog (S. cerevisiae) like 2; DIS3 mitotic control homolog like 2; FLJ36974; MGC42174; DI3L2_HUMAN.
Anti-DIS3L2抗体    
说 明 书 0.1ml  0.2ml  
研究领域 心血管  细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Sheep, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 99kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human DIS3L2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.

PubMed PubMed
产品介绍 background:
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.