|别 名||DIS3 L2; DIS3 mitotic control homolog (S. cerevisiae) like 2; DIS3 mitotic control homolog like 2; FLJ36974; MGC42174; DI3L2_HUMAN.|
|规格价格||100ul/1580元 购买 200ul/2480元 购买 大包装/询价|
|说 明 书||100ul 200ul|
|研究领域||心血管 细胞生物 免疫学|
|交叉反应||Human, Mouse, Rat, Dog, Pig, Cow, Sheep,|
|产品应用||WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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|
|性 状||Lyophilized or Liquid|
|免 疫 原||KLH conjugated synthetic peptide derived from human DIS3L2:1-100/885|
|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.|
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.
Ribonuclease that plays a critical role in RNA metabolism. It is essential for correct mitosis, and negatively regulates cell proliferation.
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.
Belongs to the RNR ribonuclease family.
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
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications