Rabbit DIS3L2 Polyclonal Antibody | anti-DIS3L2 antibody

DIS3L2 Antibody

Cat. Num. AAA154354

• Gene Names: DIS3L2; FAM6A; PRLMNS; hDIS3L2
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot
• Purity: Affinity chromatography purified via peptide column.
• Synonyms: DIS3L2; Polyclonal Antibody; DIS3L2 Antibody; DIS3 like 3'-5' exoribonuclease 2; FAM6A; PRLMNS; anti-DIS3L2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: 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.
• Purity/Purification: Affinity chromatography purified via peptide column.
• Form/Format: Liquid
• Concentration: 1 mg/mL (varies by lot)
• Sequence Length: 885

• Applicable Applications for anti-DIS3L2 antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: 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.
• Immunogen: DIS3L2 antibody was raised against a 17 amino acid peptide near the carboxy terminus of human DIS3L2
• Buffer: Supplied in PBS containing 0.02% sodium azide.
• Positive Control 1: 3T3 Cell Lysate
• Conjugate: Unconjugated
• Preparation and Storage: Store at 4 degree C for three months and -20 degree C, stable for up to one year.

Western Blot (WB)

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

Immunohistochemistry (IHC)

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

Related Product Information for anti-DIS3L2 antibody

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).

Product Categories/Family for anti-DIS3L2 antibody

Homeostasis

References

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.

NCBI and Uniprot Product Information

• NCBI GI #: 134288890
• NCBI GeneID: 129563
• NCBI Accession #: NP_689596
• NCBI GenBank Nucleotide #: NM_152383.4
• UniProt Accession #: Q8IYB7; Q53S79; Q580W6; Q5XKH0; Q69YG5; Q6AW99; Q7Z4T6; Q8N9K9
• Molecular Weight: Predicted: 97 kDa; Observed: 94 kDa
• NCBI Official Full Name: DIS3-like exonuclease 2 isoform 1
• NCBI Official Synonym Full Names: DIS3 like 3'-5' exoribonuclease 2
• NCBI Official Symbol: DIS3L2
• NCBI Official Synonym Symbols: FAM6A; PRLMNS; hDIS3L2
• NCBI Protein Information: DIS3-like exonuclease 2
• UniProt Protein Name: DIS3-like exonuclease 2
• Protein Family: DIS3-like exonuclease
• UniProt Gene Name: DIS3L2
• UniProt Synonym Gene Names: hDIS3L2
• UniProt Entry Name: DI3L2_HUMAN

NCBI Description

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]

Uniprot Description

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