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Ribonuclease T2

Known also as: RNASET2, RNASE6PL


Secreted. 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.


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


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. Ref.9

NCBI GI number(s): 156071486
Species: Homo sapiens

Links to other databases:

Database ID Link
Uniprot O00584 O00584
KEGG hsa:8635 hsa:8635
PFAM: - O00584 (Link - using uniprot id)
InterPro: - O00584 (Link - using uniprot id)
CATH: - -
SCOP: - -

Protein sequence:

Ribonuclease T2 (Homo sapiens) is product of expression of RNASET2 gene.

Ribonuclease T2 (Homo sapiens) belongs to following protein families:

Title Authors Journal Publication date (Issue) PubMed ID
A recombinant human RNASET2 glycoprotein with antitumorigenic and antiangiogenic characteristics: expression, purification, and characterization. Smirnoff P, Roiz L, Angelkovitch B, Schwartz B, Shoseyov O Cancer 2006-12-15 (107) 17109444
Characterization of RNASET2, the first human member of the Rh/T2/S family of glycoproteins. Campomenosi P, Salis S, Lindqvist C, Mariani D, Nordstrom T, Acquati F, Taramelli R Arch Biochem Biophys 2006-05-15 (449) 16620762
Tumor and metastasis suppression by the human RNASET2 gene. Acquati F, Possati L, Ferrante L, Campomenosi P, Talevi S, Bardelli S, Margiotta C, Russo A, Bortoletto E, Rocchetti R, Calza R, Cinquetti R, Monti L, Salis S, Barbanti-Brodano G, Taramelli R Int J Oncol 2005-05-01 (26) 15809705
Ribonucleases from T2 family. Deshpande RA, Shankar V Crit Rev Microbiol 2002-01-01 (28) 12109772
Acid ribonuclease and alkaline ribonuclease isoenzymes in plasma of patients with decreased glomerular filtration rate. Naskalski J, Anyszek T, Surdacka E, Hoyer B, Sulowicz W Clin Chem Lab Med 2004-03-01 (42) 15080560
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, K Genome Res 2004-10-01 (14) 15489334
Complete sequencing and characterization of 21,243 full-length human cDNAs. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K Nat Genet 2004-02-01 (36) 14702039
Mammalian Rh/T2/S-glycoprotein ribonuclease family genes: cloning of a human member located in a region of chromosome 6 (6q27) frequently deleted in human malignancies. Trubia M, Sessa L, Taramelli R Genomics 1997-06-01 (42) 9192857
The DNA sequence and analysis of human chromosome 6. Mungall AJ, Palmer SA, Sims SK, Edwards CA, Ashurst JL, Wilming L, Jones MC, Horton R, Hunt SE, Scott CE Nature 2003-10-23 (425) 14574404
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. Liu Y, Emilion G, Mungall AJ, Dunham I, Beck S, Le Meuth-Metzinger VG, Shelling AN, Charnock FM, Ganesan TS Oncogene 2002-02-17 (21) 11821951
RNASET2-deficient cystic leukoencephalopathy resembles congenital cytomegalovirus brain infection. Henneke M, Diekmann S, Ohlenbusch A, Kaiser J, Engelbrecht V, Kohlschutter A, Kratzner R, Madruga-Garrido M, Mayer M, Opitz L, Rodriguez D, Ruschendorf F, Schumacher J, Thiele H, Thoms S, Steinfeld R, Nurnberg P, Gartner J Nat Genet 2009-07-01 (41) 19525954

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Last modification of this entry: Sept. 25, 2012.

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