DIS3L2, Polyclonal Antibody

DIS3 like 3'-5' exoribonuclease 2, FAM6A, PRLMNS

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

DIS3L2 antibody is human, mouse and rat reactive. At least three isoforms of DIS3L2 are known to exist; this antibody will only detect the longest isoform. DIS3L2 is predicted to not cross-react with DIS3 or DIS3L.

Affinity chromatography purified via peptide column.

Liquid

1 mg/mL (lot specific)

Store at 4 degree C for three months and -20 degree C, stable for up to one year.

Small volumes of anti-DIS3L2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The exosome is involved in a multitude of cellular RNA processing and degradation events (1). DIS3, also known as exosome complex exonuclease RRP44, is a ribonuclease that acts directly in the processing, turnover, and surveillance of a large number of distinct RNA species (2). DIS3L2 is a paralog of DIS3, but unlike DIS3 is located in the cytoplasm (3). DIS3L2 defines a novel eukaryotic RNA degradation pathway and preferentially targets uridylated RNA (4). DIS3L2 mutations have also been associated with sporadic Wilms tumors (3).

Homeostasis

ELISA (EIA), Western Blot (WB)

DIS3L2 antibody can be used for detection of DIS3L2 by Western blot at 1 - 2 mug/ml. Antibody can also be used for immunohistochemistry starting at 5 mug/mL.

Western blot analysis of DIS3L2 in 3T3 cell lysate with DIS3L2 antibody at 1 μg/ml.

Immunohistochemistry of DIS3L2 in rat brain tissue with DIS3L2 antibody at 5 μg/mL.

Predicted: 97 kDa
Observed: 94 kDa

DIS3 like 3'-5' exoribonuclease 2

DIS3-like exonuclease 2

DIS3-like exonuclease 2

hDIS3L2  [Similar Products]

DI3L2_HUMAN

The protein encoded by this gene is similar in sequence to 3'/5' exonucleolytic subunits of the RNA exosome. The exosome is a large multimeric ribonucleotide complex responsible for degrading various RNA substrates. Several transcript variants, some protein-coding and some not, have been found for this gene. [provided by RefSeq, Mar 2012]

DIS3L2: 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). 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. 5 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.1.13.-; Ribonuclease; Tumor suppressor

Chromosomal Location of Human Ortholog: 2q37.1

Cellular Component: cytoplasm; nuclear exosome (RNase complex); polysome

Molecular Function: 3'-5'-exoribonuclease activity; magnesium ion binding; poly(U) binding; protein binding; ribonuclease activity

Biological Process: cell division; mitosis; mitotic sister chromatid separation; mRNA catabolic process, exonucleolytic; negative regulation of cell proliferation; rRNA catabolic process; rRNA processing; stem cell maintenance

Disease: Perlman Syndrome

Chen CY, Gherzi R, Ong SE, et al. AU binding proteins recruit the exosome to degrade ARE-containing mRNAs. Cell 2001; 107:451-64. Brouwer R, Allmang C, Raijmakers R, et al. Three novel components of the human exosome. J. Biol. Chem. 2001; 276:6177-84. Astuti D, Morris MR, Cooper WN, et al. Germline mutations in DIS3L2 cause the Perlman syndrome of overgrowth and Wilms tumor susceptibility. Nat. Genet. 2012; 44:277-84. Malecki M, Viegas SC, Carneiro T, et al. The exonuclease Dis3L2 defines a novel eukaryotic RNA degradation pathway. EMBO J. 2013; 32:1842-54.

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