RNASET2
entrez
8635
canonical
uniprot
O00584
canonical
ensembl
ENSG00000026297
resolver
ensp
ENSP00000426455
resolver
genesymbol
RNASET2
resolver
genesymbol_syn
RNASE6PL
resolver
hgnc
HGNC:21686
resolver
uniprot
B2RDA7
resolver
uniprot
E1P5C3
resolver
uniprot
Q5T8Q0
resolver
uniprot
Q8TCU2
resolver
uniprot
Q9BZ46
resolver
uniprot
Q9BZ47
resolver
uniprot_entry
RNT2_HUMAN
resolver
uniprot_sec
B2RDA7
resolver
uniprot_sec
E1P5C3
resolver
uniprot_sec
Q5T8Q0
resolver
uniprot_sec
Q8TCU2
resolver
uniprot_sec
Q9BZ46
resolver
uniprot_sec
Q9BZ47
resolver
activity_regulation
ACTIVITY REGULATION: Inhibited by Zn(2+) and Cu(2+). {ECO:0000269|PubMed:22735700}.
uniprot
proteins
amino_acid_sequence
MRPAALRGALLGCLCLALLCLGGADKRLRDNHEWKKLIMVQHWPETVCEKIQNDCRDPPDYWTIHGLWPDKSEGCNRSWPFNLEEIKDLLPEMRAYWPDVIHSFPNRSRFWKHEWEKHGTCAAQVDALNSQKKYFGRSLELYRELDLNSVLLKLGIKPSINYYQVADFKDALARVYGVIPKIQCLPPSQDEEVQTIGQIELCLTKQDQQLQNCTEPGEQPSPKQEVWLANGAAESRGLRVCEDGPVFYPPPKKTKH
uniprot
proteins
disease_involvement
DISEASE: Leukoencephalopathy, cystic, without megalencephaly (LCWM) [MIM:612951]: 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. {ECO:0000269|PubMed:19525954, ECO:0000269|PubMed:21199949}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
4.6.1.19
uniprot
proteins
function
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). {ECO:0000269|PubMed:16620762, ECO:0000269|PubMed:19525954, ECO:0000269|PubMed:22735700, ECO:0000269|PubMed:28730546, ECO:0000269|PubMed:30184494, ECO:0000269|PubMed:30385512, ECO:0000269|PubMed:31778653, ECO:0000269|PubMed:38697119}.
uniprot
proteins
mass_dalton
29481
uniprot
proteins
mutagenesis
MUTAGEN 65; /note="H->Y: Abolishes the effect on degradation of mitochondrion-associated cytosolic rRNAs."; /evidence="ECO:0000269|PubMed:30385512"; MUTAGEN 118; /note="H->Y: Abolishes the effect on degradation of mitochondrion-associated cytosolic rRNAs."; /evidence="ECO:0000269|PubMed:30385512
uniprot
proteins
protein_family
RNase T2 family
uniprot
proteins
pubmed
11821951
uniprot
proteins
pubmed
14574404
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15809705
uniprot
proteins
pubmed
16620762
uniprot
proteins
pubmed
19525954
uniprot
proteins
pubmed
21199949
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
22735700
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
28730546
uniprot
proteins
pubmed
30184494
uniprot
proteins
pubmed
30385512
uniprot
proteins
pubmed
31778653
uniprot
proteins
pubmed
38697119
uniprot
proteins
pubmed
9192857
uniprot
proteins
sequence_length
256
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Secreted {ECO:0000269|PubMed:15809705, ECO:0000269|PubMed:16620762}. Lysosome lumen {ECO:0000269|PubMed:16620762}. Endoplasmic reticulum lumen {ECO:0000269|PubMed:16620762}. Mitochondrion intermembrane space {ECO:0000269|PubMed:28730546, ECO:0000269|PubMed:30184494, ECO:0000269|PubMed:30385512}. Note=Full-length RNASET2 is found in the endoplasmic reticulum while smaller RNASET2 proteolytic products are found in the lysosome fraction. {ECO:0000269|PubMed:16620762}.
uniprot
proteins