DIS3L2 blocking peptide

DIS3L2 Peptide

Cat. Num. AAA154399

• Gene Names: DIS3L2; FAM6A; PRLMNS; hDIS3L2
• Applications: Blocking (BL)
• Synonyms: DIS3L2; DIS3L2 Peptide; DIS3 like 3'-5' exoribonuclease 2; FAM6A; PRLMNS; DIS3L2 blocking peptide

• Host: Human
• Form/Format: Liquid
• Concentration: 200 ug/mL (varies by lot)
• Sequence Length: 885

• Applicable Applications for DIS3L2 blocking peptide: Blocking (BL)
• Buffer: PBS pH 7.2 (10 mM NaH2PO4, 10 mM Na2HPO4, 130 mM NaCl) containing 0.1% bovine serum albumin and 0.02% sodium azide
• Location: 17 amino acid peptide near the carboxy terminus of human DIS3L2.
• Preparation and Storage: Store at -20 degree C, stable for one year.

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.

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: 40,480 Da
• 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