Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

LON peptidase N-terminal domain and RING finger protein 1

Gene

LONRF1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri123 – 159RING-type 1PROSITE-ProRule annotationAdd BLAST37
Zinc fingeri479 – 517RING-type 2PROSITE-ProRule annotationAdd BLAST39

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Ligandi

Metal-binding, Zinc

Enzyme and pathway databases

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

Names & Taxonomyi

Protein namesi
Recommended name:
LON peptidase N-terminal domain and RING finger protein 1
Alternative name(s):
RING finger protein 191
Gene namesi
Name:LONRF1
Synonyms:RNF191
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 8

Organism-specific databases

HGNCiHGNC:26302. LONRF1.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Pathology & Biotechi

Organism-specific databases

DisGeNETi91694.
OpenTargetsiENSG00000154359.
PharmGKBiPA128394743.

Polymorphism and mutation databases

BioMutaiLONRF1.
DMDMi257051033.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002776691 – 773LON peptidase N-terminal domain and RING finger protein 1Add BLAST773

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei431PhosphoserineBy similarity1

Keywords - PTMi

Phosphoprotein

Proteomic databases

MaxQBiQ17RB8.
PaxDbiQ17RB8.
PeptideAtlasiQ17RB8.
PRIDEiQ17RB8.

PTM databases

iPTMnetiQ17RB8.
PhosphoSitePlusiQ17RB8.

Expressioni

Gene expression databases

BgeeiENSG00000154359.
CleanExiHS_LONRF1.
ExpressionAtlasiQ17RB8. baseline and differential.
GenevisibleiQ17RB8. HS.

Organism-specific databases

HPAiHPA023584.
HPA024814.

Interactioni

Binary interactionsi

WithEntry#Exp.IntActNotes
ADAMTSL4Q6UY14-35EBI-2341787,EBI-10173507
ALAS1P131965EBI-2341787,EBI-3905054
ATG9AQ7Z3C63EBI-2341787,EBI-727146
BYSLQ138955EBI-2341787,EBI-358049
CALCOCO2Q131373EBI-2341787,EBI-739580
CCDC36Q8IYA85EBI-2341787,EBI-8638439
CRXO431864EBI-2341787,EBI-748171
EFHC2Q5JST65EBI-2341787,EBI-2349927
GORASP1Q9BQQ35EBI-2341787,EBI-2561458
GORASP2Q9H8Y85EBI-2341787,EBI-739467
IKZF2Q9UKS73EBI-2341787,EBI-3893057
IQCF2Q8IXL93EBI-2341787,EBI-10238842
KAT5Q929933EBI-2341787,EBI-399080
KCTD9Q7L2735EBI-2341787,EBI-4397613
KRT31Q153235EBI-2341787,EBI-948001
LHX4Q969G23EBI-2341787,EBI-2865388
NR4A1P227363EBI-2341787,EBI-721550
PLSCR1Q8WVK13EBI-2341787,EBI-10238872
RBPMSQ930623EBI-2341787,EBI-740322
SFNP319473EBI-2341787,EBI-476295
SPRY2O435973EBI-2341787,EBI-742487
STAU1O957935EBI-2341787,EBI-358174
STX11O755583EBI-2341787,EBI-714135
TRIM42A1L4B65EBI-2341787,EBI-10172216
UBE2L6O149336EBI-2341787,EBI-2129974
WDYHV1Q96HA83EBI-2341787,EBI-741158
XIAPP981705EBI-2341787,EBI-517127

Protein-protein interaction databases

BioGridi124867. 38 interactors.
IntActiQ17RB8. 54 interactors.
STRINGi9606.ENSP00000381298.

Structurei

3D structure databases

ProteinModelPortaliQ17RB8.
SMRiQ17RB8.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati48 – 81TPR 1Add BLAST34
Repeati212 – 244TPR 2Add BLAST33
Repeati246 – 278TPR 3Add BLAST33
Repeati279 – 312TPR 4Add BLAST34
Domaini565 – 766Lon N-terminalPROSITE-ProRule annotationAdd BLAST202

Sequence similaritiesi

Contains 1 Lon N-terminal domain.PROSITE-ProRule annotation
Contains 2 RING-type zinc fingers.PROSITE-ProRule annotation
Contains 4 TPR repeats.Curated

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri123 – 159RING-type 1PROSITE-ProRule annotationAdd BLAST37
Zinc fingeri479 – 517RING-type 2PROSITE-ProRule annotationAdd BLAST39

Keywords - Domaini

Repeat, TPR repeat, Zinc-finger

Phylogenomic databases

eggNOGiKOG4159. Eukaryota.
COG2802. LUCA.
GeneTreeiENSGT00440000033329.
HOGENOMiHOG000060199.
HOVERGENiHBG081924.
InParanoidiQ17RB8.
OMAiWPEVYFR.
OrthoDBiEOG091G050D.
PhylomeDBiQ17RB8.
TreeFamiTF327043.

Family and domain databases

Gene3Di1.25.40.10. 2 hits.
3.30.40.10. 2 hits.
InterProiIPR003111. LON_substr-bd_dom.
IPR015947. PUA-like_domain.
IPR013026. TPR-contain_dom.
IPR011990. TPR-like_helical_dom.
IPR019734. TPR_repeat.
IPR027370. Znf-RING_LisH.
IPR001841. Znf_RING.
IPR013083. Znf_RING/FYVE/PHD.
IPR017907. Znf_RING_CS.
[Graphical view]
PfamiPF02190. LON_substr_bdg. 1 hit.
PF13445. zf-RING_UBOX. 1 hit.
[Graphical view]
SMARTiSM00464. LON. 1 hit.
SM00184. RING. 2 hits.
SM00028. TPR. 4 hits.
[Graphical view]
SUPFAMiSSF48452. SSF48452. 1 hit.
SSF88697. SSF88697. 1 hit.
PROSITEiPS51787. LON_N. 1 hit.
PS50293. TPR_REGION. 1 hit.
PS00518. ZF_RING_1. 2 hits.
PS50089. ZF_RING_2. 2 hits.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

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

Isoform 1 (identifier: Q17RB8-1) [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
MSSPAVARTS PGGSREMAPA PQGRGRFWEV GGGSGHRLER AAAESERWEL
60 70 80 90 100
LLRRGELLAL GGHLKGALEA FAAALRRGAP ARPECLGALV DCLVFNYRLR
110 120 130 140 150
HGLGWSAAPV AGADGGAGGL LRCLGCRGFL SEPVTVPCGH SYCRRCLRRE
160 170 180 190 200
LRARCRLCRD RLPPATASAT DAEGTAPRPP PLAAAIAASD FRTSVVLNHL
210 220 230 240 250
AEKWFPGQRE RARAAGRLGE LLHQGRYREA LAAACEALRA EPSDLIVKIY
260 270 280 290 300
RAESYAGLQE FKAAIEDLNA VLFQLPDWPE VYFRKGKVLC DAGFLGDALQ
310 320 330 340 350
LFLQCLALDE DFAPAKLQVQ KILCDLLLPE NLKEGLKESS WSSLPCTKNR
360 370 380 390 400
PFDFHSVMEE SQSLNEPSPK QSEEIPEVTS EPVKGSLNRA QSAQSINSTE
410 420 430 440 450
MPAREDCLKR VSSEPVLSVQ EKGVLLKRKL SLLEQDVIVN EDGRNKLKKQ
460 470 480 490 500
GETPNEVCMF SLAYGDIPEE LIDVSDFECS LCMRLFFEPV TTPCGHSFCK
510 520 530 540 550
NCLERCLDHA PYCPLCKESL KEYLADRRYC VTQLLEELIV KYLPDELSER
560 570 580 590 600
KKIYDEETAE LSHLTKNVPI FVCTMAYPTV PCPLHVFEPR YRLMIRRSIQ
610 620 630 640 650
TGTKQFGMCV SDTQNSFADY GCMLQIRNVH FLPDGRSVVD TVGGKRFRVL
660 670 680 690 700
KRGMKDGYCT ADIEYLEDVK VENEDEIKNL RELHDLVYSQ ACSWFQNLRD
710 720 730 740 750
RFRSQILQHF GSMPEREENL QAAPNGPAWC WWLLAVLPVD PRYQLSVLSM
760 770
KSLKERLTKI QHILTYFSRD QSK
Length:773
Mass (Da):86,725
Last modified:September 1, 2009 - v2
Checksum:iB151BEB07CF3638B
GO
Isoform 2 (identifier: Q17RB8-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     452-462: Missing.

Show »
Length:762
Mass (Da):85,473
Checksum:iA9A06A836F433EE5
GO

Sequence cautioni

The sequence AAI17382 differs from that shown. Reason: Erroneous initiation.Curated
The sequence AAI17386 differs from that shown. Reason: Erroneous initiation.Curated
The sequence BAB85052 differs from that shown. Reason: Frameshift at position 463.Curated
The sequence BAG59741 differs from that shown. Reason: Frameshift at position 293.Curated
The sequence BAG62246 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti663I → V in BAG62246 (PubMed:14702039).Curated1
Sequence conflicti773K → E in BAG59741 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_058706265I → L.Corresponds to variant rs1139354dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_037971452 – 462Missing in isoform 2. 1 PublicationAdd BLAST11

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC123777 Genomic DNA. No translation available.
AK074329 mRNA. Translation: BAB85052.1. Frameshift.
AK297266 mRNA. Translation: BAG59741.1. Frameshift.
AK300538 mRNA. Translation: BAG62246.1. Different initiation.
BC004538 mRNA. Translation: AAH04538.2.
BC117381 mRNA. Translation: AAI17382.1. Different initiation.
BC117385 mRNA. Translation: AAI17386.1. Different initiation.
CCDSiCCDS5987.2. [Q17RB8-1]
RefSeqiNP_001316905.1. NM_001329976.1.
NP_689484.3. NM_152271.4. [Q17RB8-1]
UniGeneiHs.180178.

Genome annotation databases

EnsembliENST00000398246; ENSP00000381298; ENSG00000154359. [Q17RB8-1]
GeneIDi91694.
KEGGihsa:91694.
UCSCiuc003wwd.2. human. [Q17RB8-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC123777 Genomic DNA. No translation available.
AK074329 mRNA. Translation: BAB85052.1. Frameshift.
AK297266 mRNA. Translation: BAG59741.1. Frameshift.
AK300538 mRNA. Translation: BAG62246.1. Different initiation.
BC004538 mRNA. Translation: AAH04538.2.
BC117381 mRNA. Translation: AAI17382.1. Different initiation.
BC117385 mRNA. Translation: AAI17386.1. Different initiation.
CCDSiCCDS5987.2. [Q17RB8-1]
RefSeqiNP_001316905.1. NM_001329976.1.
NP_689484.3. NM_152271.4. [Q17RB8-1]
UniGeneiHs.180178.

3D structure databases

ProteinModelPortaliQ17RB8.
SMRiQ17RB8.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124867. 38 interactors.
IntActiQ17RB8. 54 interactors.
STRINGi9606.ENSP00000381298.

PTM databases

iPTMnetiQ17RB8.
PhosphoSitePlusiQ17RB8.

Polymorphism and mutation databases

BioMutaiLONRF1.
DMDMi257051033.

Proteomic databases

MaxQBiQ17RB8.
PaxDbiQ17RB8.
PeptideAtlasiQ17RB8.
PRIDEiQ17RB8.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000398246; ENSP00000381298; ENSG00000154359. [Q17RB8-1]
GeneIDi91694.
KEGGihsa:91694.
UCSCiuc003wwd.2. human. [Q17RB8-1]

Organism-specific databases

CTDi91694.
DisGeNETi91694.
GeneCardsiLONRF1.
HGNCiHGNC:26302. LONRF1.
HPAiHPA023584.
HPA024814.
neXtProtiNX_Q17RB8.
OpenTargetsiENSG00000154359.
PharmGKBiPA128394743.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4159. Eukaryota.
COG2802. LUCA.
GeneTreeiENSGT00440000033329.
HOGENOMiHOG000060199.
HOVERGENiHBG081924.
InParanoidiQ17RB8.
OMAiWPEVYFR.
OrthoDBiEOG091G050D.
PhylomeDBiQ17RB8.
TreeFamiTF327043.

Enzyme and pathway databases

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

Miscellaneous databases

GenomeRNAii91694.
PROiQ17RB8.

Gene expression databases

BgeeiENSG00000154359.
CleanExiHS_LONRF1.
ExpressionAtlasiQ17RB8. baseline and differential.
GenevisibleiQ17RB8. HS.

Family and domain databases

Gene3Di1.25.40.10. 2 hits.
3.30.40.10. 2 hits.
InterProiIPR003111. LON_substr-bd_dom.
IPR015947. PUA-like_domain.
IPR013026. TPR-contain_dom.
IPR011990. TPR-like_helical_dom.
IPR019734. TPR_repeat.
IPR027370. Znf-RING_LisH.
IPR001841. Znf_RING.
IPR013083. Znf_RING/FYVE/PHD.
IPR017907. Znf_RING_CS.
[Graphical view]
PfamiPF02190. LON_substr_bdg. 1 hit.
PF13445. zf-RING_UBOX. 1 hit.
[Graphical view]
SMARTiSM00464. LON. 1 hit.
SM00184. RING. 2 hits.
SM00028. TPR. 4 hits.
[Graphical view]
SUPFAMiSSF48452. SSF48452. 1 hit.
SSF88697. SSF88697. 1 hit.
PROSITEiPS51787. LON_N. 1 hit.
PS50293. TPR_REGION. 1 hit.
PS00518. ZF_RING_1. 2 hits.
PS50089. ZF_RING_2. 2 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiLONF1_HUMAN
AccessioniPrimary (citable) accession number: Q17RB8
Secondary accession number(s): B4DM29
, B4DU84, Q8TEA0, Q9BSV1
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 20, 2007
Last sequence update: September 1, 2009
Last modified: November 30, 2016
This is version 101 of the entry and version 2 of the sequence. [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 chromosome 8
    Human chromosome 8: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.