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Protein

E3 ubiquitin-protein ligase RNF183

Gene

RNF183

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

Functioni

Acts as a E3 ubiquitin ligase catalyzing the covalent attachment of ubiquitin moieties onto substrate proteins (PubMed:29507230). Triggers apoptosis in response to prolonged ER stress by mediating the polyubiquitination and subsequent proteasomal degradation of BCL2L1 (PubMed:29507230). May collaborate with FATE1 to restrain BIK protein levels thus regulating apoptotic signaling (PubMed:26567849).1 Publication1 Publication

Catalytic activityi

S-ubiquitinyl-[E2 ubiquitin-conjugating enzyme]-L-cysteine + [acceptor protein]-L-lysine = [E2 ubiquitin-conjugating enzyme]-L-cysteine + N6-ubiquitinyl-[acceptor protein]-L-lysine.1 Publication

Pathwayi: protein ubiquitination

This protein is involved in the pathway protein ubiquitination, which is part of Protein modification.1 Publication
View all proteins of this organism that are known to be involved in the pathway protein ubiquitination and in Protein modification.

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri13 – 60RING-typePROSITE-ProRule annotationAdd BLAST48

GO - Molecular functioni

  • metal ion binding Source: UniProtKB-KW
  • ubiquitin protein ligase activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionTransferase
Biological processApoptosis, Ubl conjugation pathway
LigandMetal-binding, Zinc

Enzyme and pathway databases

UniPathwayi
UPA00143

Names & Taxonomyi

Protein namesi
Recommended name:
E3 ubiquitin-protein ligase RNF183 (EC:2.3.2.271 Publication)
Gene namesi
Name:RNF183
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 9

Organism-specific databases

EuPathDBiHostDB:ENSG00000165188.13
HGNCiHGNC:28721 RNF183
neXtProtiNX_Q96D59

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

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 161Cytoplasmic1 PublicationAdd BLAST161
Transmembranei162 – 182Helical; Anchor for type IV membrane proteinSequence analysisAdd BLAST21
Topological domaini183 – 192Lumenal1 Publication10

Keywords - Cellular componenti

Endoplasmic reticulum, Golgi apparatus, Lysosome, Membrane

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi13C → A: Abolishes autoubiquitination; when associated with A-59. 1 Publication1
Mutagenesisi13C → S: Loss of autoubiquitination and reduced ubiquitination of BCL2L1; when associated with S-16. 1 Publication1
Mutagenesisi16C → S: Loss of autoubiquitination and reduced ubiquitination of BCL2L1; when associated with S-13. 1 Publication1
Mutagenesisi28K → R: Loss of autoubiquitination but no effect on ubiquitination of BCL2L1; when associated with R-101 and R-105. 1 Publication1
Mutagenesisi59C → A: Abolishes autoubiquitination; when associated with A-13. 1 Publication1
Mutagenesisi101K → R: Loss of autoubiquitination but no effect on ubiquitination of BCL2L1; when associated with R-28 and R-105. 1 Publication1
Mutagenesisi105K → R: Loss of autoubiquitination but no effect on ubiquitination of BCL2L1; when associated with R-28 and R-101. 1 Publication1

Organism-specific databases

OpenTargetsiENSG00000165188
PharmGKBiPA142671055

Polymorphism and mutation databases

BioMutaiRNF183
DMDMi74760778

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002473581 – 192E3 ubiquitin-protein ligase RNF183Add BLAST192

Post-translational modificationi

Autoubiquitinated (in vitro).2 Publications

Keywords - PTMi

Ubl conjugation

Proteomic databases

PaxDbiQ96D59
PRIDEiQ96D59
ProteomicsDBi76254

Expressioni

Tissue specificityi

Kidney and testis.1 Publication

Inductioni

Up-regulated by ER stress in an ERN1-dependent manner.1 Publication

Gene expression databases

BgeeiENSG00000165188 Expressed in 101 organ(s), highest expression level in corpus epididymis
CleanExiHS_RNF183
ExpressionAtlasiQ96D59 baseline and differential
GenevisibleiQ96D59 HS

Organism-specific databases

HPAiHPA057675

Interactioni

Subunit structurei

Interacts with FATE1 (PubMed:26567849). Interacts with SEC16A (By similarity). Interacts with BCL2L1 (PubMed:29507230).By similarity2 Publications

Binary interactionsi

Protein-protein interaction databases

BioGridi126500, 9 interactors
IntActiQ96D59, 9 interactors

Structurei

3D structure databases

ProteinModelPortaliQ96D59
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri13 – 60RING-typePROSITE-ProRule annotationAdd BLAST48

Keywords - Domaini

Transmembrane, Transmembrane helix, Zinc-finger

Phylogenomic databases

eggNOGiENOG410ITEM Eukaryota
ENOG4111Y1I LUCA
GeneTreeiENSGT00530000063798
HOGENOMiHOG000082461
HOVERGENiHBG093908
InParanoidiQ96D59
KOiK22157
OMAiCWNPFNN
OrthoDBiEOG091G0Q9E
PhylomeDBiQ96D59
TreeFamiTF337102

Family and domain databases

Gene3Di3.30.40.10, 1 hit
InterProiView protein in InterPro
IPR001841 Znf_RING
IPR013083 Znf_RING/FYVE/PHD
IPR017907 Znf_RING_CS
PfamiView protein in Pfam
PF13639 zf-RING_2, 1 hit
SMARTiView protein in SMART
SM00184 RING, 1 hit
PROSITEiView protein in PROSITE
PS00518 ZF_RING_1, 1 hit
PS50089 ZF_RING_2, 1 hit

Sequencei

Sequence statusi: Complete.

Q96D59-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAEQQGRELE AECPVCWNPF NNTFHTPKML DCCHSFCVEC LAHLSLVTPA
60 70 80 90 100
RRRLLCPLCR QPTVLASGQP VTDLPTDTAM LALLRLEPHH VILEGHQLCL
110 120 130 140 150
KDQPKSRYFL RQPQVYTLDL GPQPGGQTGP PPDTASATVS TPILIPSHHS
160 170 180 190
LRECFRNPQF RIFAYLMAVI LSVTLLLIFS IFWTKQFLWG VG
Length:192
Mass (Da):21,617
Last modified:April 16, 2014 - v2
Checksum:i51D5B5272DFC9540
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02709582A → T1 PublicationCorresponds to variant dbSNP:rs3750533Ensembl.1
Natural variantiVAR_027096114Q → R1 PublicationCorresponds to variant dbSNP:rs3750534Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AL449305 Genomic DNA No translation available.
BC013036 mRNA Translation: AAH13036.1
CCDSiCCDS43866.1
RefSeqiNP_659488.2, NM_145051.3
XP_006717015.1, XM_006716952.2
XP_011516520.1, XM_011518218.1
XP_011516521.1, XM_011518219.1
XP_011516522.1, XM_011518220.1
XP_016869757.1, XM_017014268.1
UniGeneiHs.729641

Genome annotation databases

EnsembliENST00000297894; ENSP00000417943; ENSG00000165188
ENST00000416588; ENSP00000420740; ENSG00000165188
ENST00000441031; ENSP00000417176; ENSG00000165188
ENST00000478815; ENSP00000419454; ENSG00000165188
GeneIDi138065
KEGGihsa:138065
UCSCiuc004bgz.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AL449305 Genomic DNA No translation available.
BC013036 mRNA Translation: AAH13036.1
CCDSiCCDS43866.1
RefSeqiNP_659488.2, NM_145051.3
XP_006717015.1, XM_006716952.2
XP_011516520.1, XM_011518218.1
XP_011516521.1, XM_011518219.1
XP_011516522.1, XM_011518220.1
XP_016869757.1, XM_017014268.1
UniGeneiHs.729641

3D structure databases

ProteinModelPortaliQ96D59
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi126500, 9 interactors
IntActiQ96D59, 9 interactors

Polymorphism and mutation databases

BioMutaiRNF183
DMDMi74760778

Proteomic databases

PaxDbiQ96D59
PRIDEiQ96D59
ProteomicsDBi76254

Protocols and materials databases

DNASUi138065
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000297894; ENSP00000417943; ENSG00000165188
ENST00000416588; ENSP00000420740; ENSG00000165188
ENST00000441031; ENSP00000417176; ENSG00000165188
ENST00000478815; ENSP00000419454; ENSG00000165188
GeneIDi138065
KEGGihsa:138065
UCSCiuc004bgz.4 human

Organism-specific databases

CTDi138065
EuPathDBiHostDB:ENSG00000165188.13
GeneCardsiRNF183
HGNCiHGNC:28721 RNF183
HPAiHPA057675
neXtProtiNX_Q96D59
OpenTargetsiENSG00000165188
PharmGKBiPA142671055
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410ITEM Eukaryota
ENOG4111Y1I LUCA
GeneTreeiENSGT00530000063798
HOGENOMiHOG000082461
HOVERGENiHBG093908
InParanoidiQ96D59
KOiK22157
OMAiCWNPFNN
OrthoDBiEOG091G0Q9E
PhylomeDBiQ96D59
TreeFamiTF337102

Enzyme and pathway databases

UniPathwayi
UPA00143

Miscellaneous databases

GenomeRNAii138065
PROiPR:Q96D59

Gene expression databases

BgeeiENSG00000165188 Expressed in 101 organ(s), highest expression level in corpus epididymis
CleanExiHS_RNF183
ExpressionAtlasiQ96D59 baseline and differential
GenevisibleiQ96D59 HS

Family and domain databases

Gene3Di3.30.40.10, 1 hit
InterProiView protein in InterPro
IPR001841 Znf_RING
IPR013083 Znf_RING/FYVE/PHD
IPR017907 Znf_RING_CS
PfamiView protein in Pfam
PF13639 zf-RING_2, 1 hit
SMARTiView protein in SMART
SM00184 RING, 1 hit
PROSITEiView protein in PROSITE
PS00518 ZF_RING_1, 1 hit
PS50089 ZF_RING_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiRN183_HUMAN
AccessioniPrimary (citable) accession number: Q96D59
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 25, 2006
Last sequence update: April 16, 2014
Last modified: November 7, 2018
This is version 124 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

Complete proteome, Reference proteome

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. Human chromosome 9
    Human chromosome 9: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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