DIS3L2, Polyclonal Antibody

DIS3-like exonuclease 2UniRule annotation; DIS3L2

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

Polyclonal

IgG

Rabbit

>95%, Protein G Purified

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

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.

3'-5'-exoribonuclease that specifically recognizes RNAs polyuridylated at their 3' end and mediates their degradation. Component of an exosome-independent RNA degradation pathway that mediates degradation of both mRNAs and miRNAs that have been polyuridylated by a terminal uridylyltransferase, such as ZCCHC11/TUT4. Mediates degradation of cytoplasmic mRNAs that have been deadenylated and subsequently uridylated at their 3'. Mediates degradation of uridylated pre-let-7 miRNAs, contributing to the maintenance of embryonic stem (ES) cells. Essential for correct mitosis, and negatively regulates cell proliferation.

ELISA (EIA)

40,480 Da

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: Ribonuclease; Tumor suppressor; EC 3.1.13.-

Chromosomal Location of Human Ortholog: 2q37.1

Cellular Component: cytoplasm; 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

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