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Protein

E3 ubiquitin-protein ligase RNF126

Gene

RNF126

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

Functioni

E3 ubiquitin-protein ligase that mediates ubiquitination oF target proteins (PubMed:23277564, PubMed:24275455, PubMed:24981174). Depending on the associated E2 ligase, mediates 'Lys-48'- and 'Lys-63'-linked polyubiquitination of substrates (By similarity). Part of a BAG6-dependent quality control process ensuring that proteins of the secretory pathway that are mislocalized to the cytosol are degraded by the proteasome. Probably acts by providing the ubiquitin ligase activity associated with the BAG6 complex and be responsible for ubiquitination of the hydrophobic mislocalized proteins and their targeting to the proteasome (PubMed:24981174, PubMed:29042515). May also play a role in the endosomal recycling of IGF2R, the cation-independent mannose-6-phosphate receptor (PubMed:24275455). May play a role in the endosomal sorting and degradation of several membrane receptors including EGFR, FLT3, MET and CXCR4, by mediating their ubiquitination (PubMed:23418353). By ubiquitinating CDKN1A/p21 and targeting it for degradation, may also promote cell proliferation (PubMed:23026136). May monoubiquitinate AICDA (PubMed:23277564).By similarity1 Publication5 Publications

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.2 Publications

Pathwayi: protein ubiquitination

This protein is involved in the pathway protein ubiquitination, which is part of Protein modification.2 Publications
View all proteins of this organism that are known to be involved in the pathway protein ubiquitination and in Protein modification.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi13ZincCombined sources1 Publication1
Metal bindingi16ZincCombined sources1 Publication1
Metal bindingi29ZincCombined sources1 Publication1
Metal bindingi32ZincCombined sources1 Publication1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri13 – 32C4-type1 PublicationAdd BLAST20
Zinc fingeri229 – 270RING-typePROSITE-ProRule annotationAdd BLAST42

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionTransferase
Biological processUbl conjugation pathway
LigandMetal-binding, Zinc

Enzyme and pathway databases

ReactomeiR-HSA-983168 Antigen processing: Ubiquitination & Proteasome degradation
UniPathwayi
UPA00143

Names & Taxonomyi

Protein namesi
Recommended name:
E3 ubiquitin-protein ligase RNF126Curated (EC:2.3.2.272 Publications)
Alternative name(s):
RING finger protein 126Imported
Gene namesi
Name:RNF126Imported
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000070423.17
HGNCiHGNC:21151 RNF126
MIMi615177 gene
neXtProtiNX_Q9BV68

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

Cytoplasm, Nucleus

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi14H → A: Impaired interaction with BAG6. 1 Publication1
Mutagenesisi36F → A: Impaired interaction with BAG6. 1 Publication1
Mutagenesisi38 – 39EE → RR: Impaired interaction with BAG6. 1 Publication2
Mutagenesisi229C → A: Loss of E3 ligase activity; when associated with A-232. 1 Publication1
Mutagenesisi232C → A: Loss of E3 ligase activity; when associated with A-229. 1 Publication1

Organism-specific databases

OpenTargetsiENSG00000070423
PharmGKBiPA134876469

Polymorphism and mutation databases

BioMutaiRNF126
DMDMi74762712

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00000560932 – 326E3 ubiquitin-protein ligase RNF126Add BLAST325

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1
Modified residuei5PhosphoserineCombined sources1

Post-translational modificationi

Ubiquitinated. May undergo autoubiquitination.1 Publication

Keywords - PTMi

Acetylation, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiQ9BV68
MaxQBiQ9BV68
PaxDbiQ9BV68
PeptideAtlasiQ9BV68
PRIDEiQ9BV68
ProteomicsDBi79173
79174 [Q9BV68-2]

PTM databases

iPTMnetiQ9BV68
PhosphoSitePlusiQ9BV68

Expressioni

Tissue specificityi

Highly expressed in liver and testis.1 Publication

Gene expression databases

BgeeiENSG00000070423 Expressed in 213 organ(s), highest expression level in skin of abdomen
CleanExiHS_RNF126
ExpressionAtlasiQ9BV68 baseline and differential
GenevisibleiQ9BV68 HS

Organism-specific databases

HPAiHPA043050

Interactioni

Subunit structurei

Interacts with CCDC50, EGFR, FLT3 and SCAMP3 (PubMed:23418353). Interacts with BAG6 (via ubiquitin-like domain); required for BAG6-dependent ubiquitination of proteins mislocalized to the cytosol (PubMed:24981174, PubMed:27193484). Interacts with CDKN1A (PubMed:23026136). Interacts with AICDA (PubMed:23277564).5 Publications

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi120790, 103 interactors
IntActiQ9BV68, 31 interactors
MINTiQ9BV68
STRINGi9606.ENSP00000292363

Structurei

Secondary structure

1326
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliQ9BV68
SMRiQ9BV68
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni5 – 100Required for interaction with BAG61 PublicationAdd BLAST96
Regioni200 – 304Sufficient for interaction with AICDA1 PublicationAdd BLAST105

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi289 – 303Ser-richAdd BLAST15

Domaini

The C4-type zinc finger is required for interaction with BAG6.1 Publication

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri13 – 32C4-type1 PublicationAdd BLAST20
Zinc fingeri229 – 270RING-typePROSITE-ProRule annotationAdd BLAST42

Keywords - Domaini

Zinc-finger

Phylogenomic databases

eggNOGiKOG0800 Eukaryota
ENOG41121N2 LUCA
GeneTreeiENSGT00530000062967
HOGENOMiHOG000116417
HOVERGENiHBG059832
InParanoidiQ9BV68
KOiK11982
OMAiWGMLHSN
OrthoDBiEOG091G0SOO
PhylomeDBiQ9BV68
TreeFamiTF317985

Family and domain databases

Gene3Di3.30.40.10, 1 hit
InterProiView protein in InterPro
IPR039525 RNF126-like_zinc-ribbon
IPR001841 Znf_RING
IPR013083 Znf_RING/FYVE/PHD
PfamiView protein in Pfam
PF13639 zf-RING_2, 1 hit
PF14369 zinc_ribbon_9, 1 hit
SMARTiView protein in SMART
SM00184 RING, 1 hit
PROSITEiView protein in PROSITE
PS50089 ZF_RING_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

This entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q9BV68-1) [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
MAEASPHPGR YFCHCCSVEI VPRLPDYICP RCESGFIEEL PEETRSTENG
60 70 80 90 100
SAPSTAPTDQ SRPPLEHVDQ HLFTLPQGYG QFAFGIFDDS FEIPTFPPGA
110 120 130 140 150
QADDGRDPES RRERDHPSRH RYGARQPRAR LTTRRATGRH EGVPTLEGII
160 170 180 190 200
QQLVNGIITP ATIPSLGPWG VLHSNPMDYA WGANGLDAII TQLLNQFENT
210 220 230 240 250
GPPPADKEKI QALPTVPVTE EHVGSGLECP VCKDDYALGE RVRQLPCNHL
260 270 280 290 300
FHDGCIVPWL EQHDSCPVCR KSLTGQNTAT NPPGLTGVSF SSSSSSSSSS
310 320
SPSNENATWS PLGRPQPPRP LSNLTL
Length:326
Mass (Da):35,585
Last modified:June 1, 2001 - v1
Checksum:iD422FFB090362F46
GO
Isoform 2 (identifier: Q9BV68-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     309-311: WSP → SNS
     312-326: Missing.

Show »
Length:311
Mass (Da):33,861
Checksum:i0C748E78E6719C3E
GO

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
K7EIY6K7EIY6_HUMAN
E3 ubiquitin-protein ligase RNF126
RNF126
283Annotation score:
U3KQF4U3KQF4_HUMAN
E3 ubiquitin-protein ligase RNF126
RNF126
74Annotation score:

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05721768V → M. Corresponds to variant dbSNP:rs2285751Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_015693309 – 311WSP → SNS in isoform 2. 2 Publications3
Alternative sequenceiVSP_015694312 – 326Missing in isoform 2. 2 PublicationsAdd BLAST15

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK000559 mRNA Translation: BAA91254.1
BC001442 mRNA Translation: AAH01442.1
BC025374 mRNA Translation: AAH25374.1
CCDSiCCDS12039.1 [Q9BV68-2]
RefSeqiNP_919442.1, NM_194460.2 [Q9BV68-2]
UniGeneiHs.69554

Genome annotation databases

EnsembliENST00000292363; ENSP00000292363; ENSG00000070423 [Q9BV68-2]
GeneIDi55658
KEGGihsa:55658
UCSCiuc010drs.4 human [Q9BV68-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK000559 mRNA Translation: BAA91254.1
BC001442 mRNA Translation: AAH01442.1
BC025374 mRNA Translation: AAH25374.1
CCDSiCCDS12039.1 [Q9BV68-2]
RefSeqiNP_919442.1, NM_194460.2 [Q9BV68-2]
UniGeneiHs.69554

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2N9ONMR-A1-40[»]
2N9PNMR-A1-40[»]
ProteinModelPortaliQ9BV68
SMRiQ9BV68
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi120790, 103 interactors
IntActiQ9BV68, 31 interactors
MINTiQ9BV68
STRINGi9606.ENSP00000292363

PTM databases

iPTMnetiQ9BV68
PhosphoSitePlusiQ9BV68

Polymorphism and mutation databases

BioMutaiRNF126
DMDMi74762712

Proteomic databases

EPDiQ9BV68
MaxQBiQ9BV68
PaxDbiQ9BV68
PeptideAtlasiQ9BV68
PRIDEiQ9BV68
ProteomicsDBi79173
79174 [Q9BV68-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000292363; ENSP00000292363; ENSG00000070423 [Q9BV68-2]
GeneIDi55658
KEGGihsa:55658
UCSCiuc010drs.4 human [Q9BV68-1]

Organism-specific databases

CTDi55658
EuPathDBiHostDB:ENSG00000070423.17
GeneCardsiRNF126
H-InvDBiHIX0014558
HGNCiHGNC:21151 RNF126
HPAiHPA043050
MIMi615177 gene
neXtProtiNX_Q9BV68
OpenTargetsiENSG00000070423
PharmGKBiPA134876469
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0800 Eukaryota
ENOG41121N2 LUCA
GeneTreeiENSGT00530000062967
HOGENOMiHOG000116417
HOVERGENiHBG059832
InParanoidiQ9BV68
KOiK11982
OMAiWGMLHSN
OrthoDBiEOG091G0SOO
PhylomeDBiQ9BV68
TreeFamiTF317985

Enzyme and pathway databases

UniPathwayi
UPA00143

ReactomeiR-HSA-983168 Antigen processing: Ubiquitination & Proteasome degradation

Miscellaneous databases

ChiTaRSiRNF126 human
GenomeRNAii55658
PROiPR:Q9BV68
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000070423 Expressed in 213 organ(s), highest expression level in skin of abdomen
CleanExiHS_RNF126
ExpressionAtlasiQ9BV68 baseline and differential
GenevisibleiQ9BV68 HS

Family and domain databases

Gene3Di3.30.40.10, 1 hit
InterProiView protein in InterPro
IPR039525 RNF126-like_zinc-ribbon
IPR001841 Znf_RING
IPR013083 Znf_RING/FYVE/PHD
PfamiView protein in Pfam
PF13639 zf-RING_2, 1 hit
PF14369 zinc_ribbon_9, 1 hit
SMARTiView protein in SMART
SM00184 RING, 1 hit
PROSITEiView protein in PROSITE
PS50089 ZF_RING_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiRN126_HUMAN
AccessioniPrimary (citable) accession number: Q9BV68
Secondary accession number(s): Q9NWX1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 27, 2005
Last sequence update: June 1, 2001
Last modified: October 10, 2018
This is version 145 of the entry and version 1 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

Documents

  1. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  2. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
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