RNASET2 Has ribonuclease activity, with higher activity at acidic pH. May play a role in cellular RNA catabolism. Defects in RNASET2 are the cause of 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. Belongs to the RNase T2 family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC 3.1.27.-; RNA-binding; Ribonuclease; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 6q27
Cellular Component:  endoplasmic reticulum lumen; extracellular region; extracellular space; lysosomal lumen; lysosome
Molecular Function:  endoribonuclease activity; ribonuclease activity; ribonuclease T2 activity; RNA binding
Biological Process:  neutrophil degranulation; RNA catabolic process; RNA phosphodiester bond hydrolysis, endonucleolytic
Disease: Leukoencephalopathy, Cystic, Without Megalencephaly
Reference #:  O00584 (UniProtKB)
Alt. Names/Synonyms: bA514O12.3; FLJ42372; Ribonuclease 6; Ribonuclease T2; RNASE6PL; RNASET2; RNT2
Gene Symbols: RNASET2
Molecular weight: 29,481 Da
Basal Isoelectric point: 6.66  Predict pI for various phosphorylation states
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RNASET2

Protein Structure Not Found.


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