UniProt ID: B8XY56 |
FUNCTION: Has ribonuclease activity, with higher activity at acidic pH. Probably is involved in lysosomal degradation of ribosomal RNA. {ECO:0000269|PubMed:21199949}. CATALYTIC ACTIVITY: Reaction=a ribonucleotidyl-ribonucleotide-RNA + H2O = a 3'-end 3'- phospho-ribonucleotide-RNA + a 5'-end dephospho-ribonucleoside-RNA + H(+); Xref=Rhea:RHEA:68052, Rhea:RHEA-COMP:10463, Rhea:RHEA- COMP:13936, Rhea:RHEA-COMP:17355, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:83062, ChEBI:CHEBI:138284, ChEBI:CHEBI:173118; EC=4.6.1.19; Evidence={ECO:0000255|PROSITE- ProRule:PRU10045}; SUBCELLULAR LOCATION: Lysosome lumen {ECO:0000250}. Endoplasmic reticulum lumen {ECO:0000250}. Secreted {ECO:0000250}. TISSUE SPECIFICITY: Ubiquitous. {ECO:0000269|PubMed:19619322}. DISRUPTION PHENOTYPE: AO127 mutants were identified based on increased acridine orange uptake. AO127 mutants display enlarged lysosomes in brain and lysosomal accumulation of undigested ribosomal RNA in brain neurons, white matter lesions adjacent to brain ventricles, and frequent focal white matter anomalies throughout the brain. Nevertheless, mutants appear basically normal, reach normal adult size and length, are fertile and do not display obvious gross motor deficiencies. {ECO:0000269|PubMed:21199949}. SIMILARITY: Belongs to the RNase T2 family. {ECO:0000305}. |
UniProt ID: A0A8M9PY62 |
SUBCELLULAR LOCATION: Lysosome lumen {ECO:0000256|ARBA:ARBA00004227}. SIMILARITY: Belongs to the RNase T2 family. {ECO:0000256|ARBA:ARBA00007469, ECO:0000256|RuleBase:RU004328}. |
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