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Protein

Ribonuclease T2

Gene

RNASET2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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.By similarity3 Publications

Enzyme regulationi

Inhibited by Zn2+ and Cu2+.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei65By similarity1
Active sitei114By similarity1
Active sitei118By similarity1

GO - Molecular functioni

  • ribonuclease activity Source: UniProtKB
  • ribonuclease T2 activity Source: InterPro
  • RNA binding Source: InterPro

GO - Biological processi

  • neutrophil degranulation Source: Reactome
  • RNA catabolic process Source: UniProtKB

Keywordsi

Molecular functionEndonuclease, Hydrolase, Nuclease

Enzyme and pathway databases

ReactomeiR-HSA-6798695 Neutrophil degranulation

Names & Taxonomyi

Protein namesi
Recommended name:
Ribonuclease T2 (EC:3.1.27.-)
Alternative name(s):
Ribonuclease 6
Gene namesi
Name:RNASET2
Synonyms:RNASE6PL
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

EuPathDBiHostDB:ENSG00000026297.15
HGNCiHGNC:21686 RNASET2
MIMi612944 gene
neXtProtiNX_O00584

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Endoplasmic reticulum, Lysosome, Secreted

Pathology & Biotechi

Involvement in diseasei

Leukoencephalopathy, cystic, without megalencephaly (LCWM)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn 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.
See also OMIM:612951
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_063596184C → R in LCWM; the loss of a disulfide bond may affect protein folding and stability; the protein is retained in the endoplasmic reticulum. 2 PublicationsCorresponds to variant dbSNP:rs121918137EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi8635
MalaCardsiRNASET2
MIMi612951 phenotype
OpenTargetsiENSG00000026297
Orphaneti85136 Cystic leukoencephalopathy without megalencephaly
PharmGKBiPA128394541

Polymorphism and mutation databases

BioMutaiRNASET2

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 24Sequence analysisAdd BLAST24
ChainiPRO_000003098725 – 256Ribonuclease T2Add BLAST232

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi48 ↔ 551 Publication
Disulfide bondi75 ↔ 1211 Publication
Glycosylationi76N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi106N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi184 ↔ 2411 Publication
Disulfide bondi202 ↔ 2131 Publication
Glycosylationi212N-linked (GlcNAc...) asparagine1 Publication1

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

EPDiO00584
MaxQBiO00584
PaxDbiO00584
PeptideAtlasiO00584
PRIDEiO00584

PTM databases

iPTMnetiO00584
PhosphoSitePlusiO00584
SwissPalmiO00584

Expressioni

Tissue specificityi

Ubiquitous. Higher expression levels observed in the temporal lobe and fetal brain.1 Publication

Gene expression databases

BgeeiENSG00000026297
CleanExiHS_RNASET2
ExpressionAtlasiO00584 baseline and differential
GenevisibleiO00584 HS

Organism-specific databases

HPAiHPA029013
HPA066509

Interactioni

Protein-protein interaction databases

BioGridi114188, 12 interactors
IntActiO00584, 5 interactors
STRINGi9606.ENSP00000422846

Structurei

Secondary structure

1256
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi36 – 42Combined sources7
Helixi44 – 47Combined sources4
Beta strandi50 – 52Combined sources3
Helixi53 – 55Combined sources3
Beta strandi63 – 71Combined sources9
Helixi83 – 89Combined sources7
Helixi90 – 96Combined sources7
Helixi108 – 117Combined sources10
Helixi119 – 122Combined sources4
Helixi126 – 128Combined sources3
Helixi131 – 145Combined sources15
Helixi147 – 153Combined sources7
Helixi165 – 176Combined sources12
Beta strandi181 – 185Combined sources5
Beta strandi195 – 204Combined sources10
Turni205 – 207Combined sources3
Beta strandi243 – 247Combined sources5

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3T0OX-ray1.59A25-256[»]
ProteinModelPortaliO00584
SMRiO00584
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the RNase T2 family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG1642 Eukaryota
ENOG4111M7G LUCA
GeneTreeiENSGT00640000091563
HOVERGENiHBG050037
InParanoidiO00584
KOiK01166
PhylomeDBiO00584
TreeFamiTF315063

Family and domain databases

CDDicd01061 RNase_T2_euk, 1 hit
Gene3Di3.90.730.10, 1 hit
InterProiView protein in InterPro
IPR033697 Ribonuclease_T2_eukaryotic
IPR001568 RNase_T2-like
IPR036430 RNase_T2-like_sf
IPR018188 RNase_T2_His_AS_1
IPR033130 RNase_T2_His_AS_2
PANTHERiPTHR11240 PTHR11240, 1 hit
PfamiView protein in Pfam
PF00445 Ribonuclease_T2, 1 hit
SUPFAMiSSF55895 SSF55895, 1 hit
PROSITEiView protein in PROSITE
PS00530 RNASE_T2_1, 1 hit
PS00531 RNASE_T2_2, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: O00584-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MRPAALRGAL LGCLCLALLC LGGADKRLRD NHEWKKLIMV QHWPETVCEK
60 70 80 90 100
IQNDCRDPPD YWTIHGLWPD KSEGCNRSWP FNLEEIKDLL PEMRAYWPDV
110 120 130 140 150
IHSFPNRSRF WKHEWEKHGT CAAQVDALNS QKKYFGRSLE LYRELDLNSV
160 170 180 190 200
LLKLGIKPSI NYYQVADFKD ALARVYGVIP KIQCLPPSQD EEVQTIGQIE
210 220 230 240 250
LCLTKQDQQL QNCTEPGEQP SPKQEVWLAN GAAESRGLRV CEDGPVFYPP

PKKTKH
Length:256
Mass (Da):29,481
Last modified:November 1, 1999 - v2
Checksum:i7C8BB08B8ED853EB
GO
Isoform 2 (identifier: O00584-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     88-121: DLLPEMRAYWPDVIHSFPNRSRFWKHEWEKHGTC → KNWMEITDSSLPSPSTLPIINIFYSVLHLLQLMN
     122-256: Missing.

Note: May be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay.
Show »
Length:121
Mass (Da):14,000
Checksum:i984FAD634B7286E8
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_063596184C → R in LCWM; the loss of a disulfide bond may affect protein folding and stability; the protein is retained in the endoplasmic reticulum. 2 PublicationsCorresponds to variant dbSNP:rs121918137EnsemblClinVar.1
Natural variantiVAR_013004236R → W. Corresponds to variant dbSNP:rs11159EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_00840588 – 121DLLPE…KHGTC → KNWMEITDSSLPSPSTLPII NIFYSVLHLLQLMN in isoform 2. 1 PublicationAdd BLAST34
Alternative sequenceiVSP_008406122 – 256Missing in isoform 2. 1 PublicationAdd BLAST135

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U85625 mRNA Translation: AAC51363.2
AJ419865 mRNA Translation: CAD12030.1
AJ419866 mRNA Translation: CAD12031.1
AK315467 mRNA Translation: BAG37854.1
AL133458 Genomic DNA No translation available.
AL159163 Genomic DNA No translation available.
CH471051 Genomic DNA Translation: EAW47512.1
CH471051 Genomic DNA Translation: EAW47513.1
CH471051 Genomic DNA Translation: EAW47514.1
BC001660 mRNA Translation: AAH01660.1
BC001819 mRNA Translation: AAH01819.1
BC039713 mRNA Translation: AAH39713.1
BC051912 mRNA Translation: AAH51912.1
CCDSiCCDS5295.1 [O00584-1]
PIRiS78046
RefSeqiNP_003721.2, NM_003730.4 [O00584-1]
UniGeneiHs.529989

Genome annotation databases

EnsembliENST00000421787; ENSP00000390833; ENSG00000026297 [O00584-2]
ENST00000476238; ENSP00000422846; ENSG00000026297 [O00584-1]
ENST00000508775; ENSP00000426455; ENSG00000026297 [O00584-1]
GeneIDi8635
KEGGihsa:8635
UCSCiuc003qve.4 human [O00584-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiRNT2_HUMAN
AccessioniPrimary (citable) accession number: O00584
Secondary accession number(s): B2RDA7
, E1P5C3, Q5T8Q0, Q8TCU2, Q9BZ46, Q9BZ47
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 3, 2002
Last sequence update: November 1, 1999
Last modified: March 28, 2018
This is version 155 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

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