ID:RNT2_HUMAN DESCRIPTION: RecName: Full=Ribonuclease T2; EC=3.1.27.-; AltName: Full=Ribonuclease 6; Flags: Precursor; FUNCTION: Has ribonuclease activity, with higher activity at acidic pH. Probably is involved in lysosomal degradation of ribosomal RNA (By similarity). Probably plays a role in cellular RNA catabolism. ENZYME REGULATION: Inhibited by Zn(2+) and Cu(2+). SUBCELLULAR LOCATION: Secreted. Lysosome lumen. Endoplasmic reticulum lumen. Note=Subcellular fractionation of transfected ovarian cancer cells reveals full-length RNASET2 in the endoplasmic reticulum fraction and the 2 smaller RNASET2 proteolytic products in the lysosome fraction. TISSUE SPECIFICITY: Ubiquitous. Higher expression levels observed in the temporal lobe and fetal brain. DISEASE: Defects in RNASET2 are the cause of 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. SIMILARITY: Belongs to the RNase T2 family. WEB RESOURCE: Name=Atlas of Genetics and Cytogenetics in Oncology and Haematology; URL="http://atlasgeneticsoncology.org/Genes/RNASET2ID518ch6q27.html";
ModBase Predicted Comparative 3D Structure on O00584
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