Recombinant Human Ribonuclease T2 (RNASET2)

Function

Ribonuclease that plays an essential role in innate immune response by recognizing and degrading RNAs from microbial pathogens that are subsequently sensed by TLR8 (PubMed:31778653). Cleaves preferentially single-stranded RNA molecules between purine and uridine residues, which critically contributes to the supply of catabolic uridine and the generation of purine-2',3'-cyclophosphate-terminated oligoribonucleotides (PubMed:31778653, PubMed:38697119). In turn, RNase T2 degradation products promote the RNA-dependent activation of TLR8 (PubMed:31778653). In plasmacytoid dendritic cells, it cooperates with PLD3 or PLD4 5'->3' exonucleases to process RNA fragments and release 2',3'-cyclic guanosine monophosphate (2',3'-cGMP), a potent stimulatory ligand for TLR7 (PubMed:38697119). Also plays a key role in degradation of mitochondrial RNA and processing of non-coding RNA imported from the cytosol into mitochondria (PubMed:28730546, PubMed:30184494). Participates as well in degradation of mitochondrion-associated cytosolic rRNAs (PubMed:30385512)

Subcellular Location

Secreted; Lysosome lumen; Endoplasmic reticulum lumen; Mitochondrion intermembrane space

Disease Association

Leukoencephalopathy, cystic, without megalencephaly (LCWM) : An infantile-onset syndrome of cerebral leukoencephalopathy. Affected newborns develop microcephaly and neurologic abnormalities including psychomotor impairment, seizures and sensorineural hearing impairment. The brain shows multifocal white matter lesions, anterior temporal lobe subcortical cysts, pericystic abnormal myelination, ventriculomegaly and intracranial calcifications. [The disease is caused by variants affecting the gene represented in this entry]

Tissue Specificity

Ubiquitous. Higher expression levels observed in the temporal lobe and fetal brain

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• Liu Y. et al. Physical and transcript map of the region between D6S264 and D6S149 on chromosome 6q27, the minimal region of allele loss in sporadic epithelial ovarian cancer. — Oncogene (2002)
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• Mungall A.J. et al. The DNA sequence and analysis of human chromosome 6. — Nature (2003)
• Mural R.J. et al. PubMed: CI-5GBD0VIIJ7C63 (SEP-)
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). — Genome Res. (2004)
• Acquati F. et al. Tumor and metastasis suppression by the human RNASET2 gene. — Int. J. Oncol. (2005)
• Campomenosi P. et al. Characterization of RNASET2, the first human member of the Rh/T2/S family of glycoproteins. — Arch. Biochem. Biophys. (2006)