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Protein

Derlin-2

Gene

DERL2

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

Functioni

Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal glycoproteins, but not that of misfolded nonglycoproteins. May act by forming a channel that allows the retrotranslocation of misfolded glycoproteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and misfolded glycoproteins (PubMed:16186509, PubMed:16449189). May also be involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the endoplasmic reticulum and reroutes them to the cytosol for proteasomal degradation (PubMed:26565908).3 Publications
(Microbial infection) In contrast to DERL1, it is not involved in the degradation of MHC class I heavy chains following infection by cytomegaloviruses.1 Publication

GO - Biological processi

Keywordsi

Biological processUnfolded protein response

Enzyme and pathway databases

ReactomeiR-HSA-382556 ABC-family proteins mediated transport
R-HSA-5358346 Hedgehog ligand biogenesis
R-HSA-5362768 Hh mutants that don't undergo autocatalytic processing are degraded by ERAD
R-HSA-5678895 Defective CFTR causes cystic fibrosis
R-HSA-901032 ER Quality Control Compartment (ERQC)

Protein family/group databases

TCDBi3.A.16.1.4 the endoplasmic reticular retrotranslocon (er-rt) family

Names & Taxonomyi

Protein namesi
Recommended name:
Derlin-21 Publication
Alternative name(s):
Degradation in endoplasmic reticulum protein 2
Short name:
DERtrin-2
Der1-like protein 21 Publication
F-LAN-1Imported
F-LANa1 Publication
Gene namesi
Name:DERL2Imported
Synonyms:DER2, FLANA1 Publication
ORF Names:CGI-101Imported, SBBI53Imported
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 17

Organism-specific databases

EuPathDBiHostDB:ENSG00000072849.10
HGNCiHGNC:17943 DERL2
MIMi610304 gene
neXtProtiNX_Q9GZP9

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 – 57CytoplasmicSequence analysisAdd BLAST57
Transmembranei58 – 78Helical; Name=1Sequence analysisAdd BLAST21
Topological domaini79 – 96LumenalSequence analysisAdd BLAST18
Transmembranei97 – 117Helical; Name=2Sequence analysisAdd BLAST21
Topological domaini118 – 150CytoplasmicSequence analysisAdd BLAST33
Transmembranei151 – 171Helical; Name=3Sequence analysisAdd BLAST21
Topological domaini172LumenalSequence analysis1
Transmembranei173 – 193Helical; Name=4Sequence analysisAdd BLAST21
Topological domaini194 – 239CytoplasmicSequence analysisAdd BLAST46

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi51009
OpenTargetsiENSG00000072849
PharmGKBiPA134896343

Polymorphism and mutation databases

BioMutaiDERL2
DMDMi50400648

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002190451 – 239Derlin-2Add BLAST239

Proteomic databases

EPDiQ9GZP9
MaxQBiQ9GZP9
PaxDbiQ9GZP9
PeptideAtlasiQ9GZP9
PRIDEiQ9GZP9
ProteomicsDBi80110

PTM databases

iPTMnetiQ9GZP9
PhosphoSitePlusiQ9GZP9

Expressioni

Tissue specificityi

Ubiquitous. Overexpressed in various hepatocarcinomas.2 Publications

Inductioni

Up-regulated in response to endoplasmic reticulum stress via the ERN1-XBP1 pathway of the unfolded protein response (UPR).1 Publication

Gene expression databases

BgeeiENSG00000072849 Expressed in 220 organ(s), highest expression level in right testis
CleanExiHS_DERL2
ExpressionAtlasiQ9GZP9 baseline and differential
GenevisibleiQ9GZP9 HS

Organism-specific databases

HPAiCAB037287
HPA048957
HPA056551

Interactioni

Subunit structurei

Forms homo- and heterooligomers with DERL3 and, to a lesser extent, with DERL1 (PubMed:16186509). Interacts with the SEL1L/SYVN1 and VCP/SELENOS protein complexes (PubMed:16186509). Mediates association between VCP and EDEM1, as well as that between VCP and the misfolded glycoproteins (PubMed:16449189). Interacts with OS9 (PubMed:19084021). Interacts with SELENOK and SELENOS (PubMed:22016385). Interacts with the signal recognition particle/SRP and the SRP receptor; in the process of endoplasmic reticulum stress-induced pre-emptive quality control (PubMed:26565908).5 Publications

Binary interactionsi

Protein-protein interaction databases

BioGridi119216, 51 interactors
CORUMiQ9GZP9
IntActiQ9GZP9, 34 interactors
MINTiQ9GZP9
STRINGi9606.ENSP00000158771

Structurei

3D structure databases

ProteinModelPortaliQ9GZP9
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the derlin family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0858 Eukaryota
COG5291 LUCA
GeneTreeiENSGT00530000063156
HOGENOMiHOG000200948
HOVERGENiHBG051338
InParanoidiQ9GZP9
KOiK13989
OMAiWWRRLFE
OrthoDBiEOG091G0IPJ
PhylomeDBiQ9GZP9
TreeFamiTF314715

Family and domain databases

InterProiView protein in InterPro
IPR007599 DER1
PANTHERiPTHR11009 PTHR11009, 1 hit
PfamiView protein in Pfam
PF04511 DER1, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

This entry has 1 described isoform and 7 potential isoforms that are computationally mapped.Show allAlign All

Q9GZP9-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAYQSLRLEY LQIPPVSRAY TTACVLTTAA VQLELITPFQ LYFNPELIFK
60 70 80 90 100
HFQIWRLITN FLFFGPVGFN FLFNMIFLYR YCRMLEEGSF RGRTADFVFM
110 120 130 140 150
FLFGGFLMTL FGLFVSLVFL GQAFTIMLVY VWSRRNPYVR MNFFGLLNFQ
160 170 180 190 200
APFLPWVLMG FSLLLGNSII VDLLGIAVGH IYFFLEDVFP NQPGGIRILK
210 220 230
TPSILKAIFD TPDEDPNYNP LPEERPGGFA WGEGQRLGG
Length:239
Mass (Da):27,567
Last modified:March 1, 2001 - v1
Checksum:iCAA228487C3CCB5C
GO

Computationally mapped potential isoform sequencesi

There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
I3L1S8I3L1S8_HUMAN
Derlin
DERL2
144Annotation score:
I3L1T3I3L1T3_HUMAN
Derlin
DERL2
158Annotation score:
I3L0R8I3L0R8_HUMAN
Derlin
DERL2
200Annotation score:
I3L1W3I3L1W3_HUMAN
Derlin
DERL2
103Annotation score:
I3L4W7I3L4W7_HUMAN
Derlin-2
DERL2
79Annotation score:
K7EQE8K7EQE8_HUMAN
Derlin-2
DERL2
82Annotation score:
I3L3R8I3L3R8_HUMAN
Derlin-2
DERL2
74Annotation score:

Sequence cautioni

The sequence AAD34096 differs from that shown. Reason: Frameshift at positions 122, 128 and 136.Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF132289 mRNA Translation: AAG43049.1
AF208065 mRNA Translation: AAL14869.1
AF151859 mRNA Translation: AAD34096.1 Frameshift.
AF242523 mRNA Translation: AAF99603.1
CR457202 mRNA Translation: CAG33483.1
BC010890 mRNA Translation: AAH10890.1
CCDSiCCDS11073.1
PIRiJC7752
RefSeqiNP_001291708.1, NM_001304779.1
NP_057125.2, NM_016041.4
UniGeneiHs.286131
Hs.730726

Genome annotation databases

EnsembliENST00000158771; ENSP00000158771; ENSG00000072849
GeneIDi51009
KEGGihsa:51009
UCSCiuc002gcc.2 human

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF132289 mRNA Translation: AAG43049.1
AF208065 mRNA Translation: AAL14869.1
AF151859 mRNA Translation: AAD34096.1 Frameshift.
AF242523 mRNA Translation: AAF99603.1
CR457202 mRNA Translation: CAG33483.1
BC010890 mRNA Translation: AAH10890.1
CCDSiCCDS11073.1
PIRiJC7752
RefSeqiNP_001291708.1, NM_001304779.1
NP_057125.2, NM_016041.4
UniGeneiHs.286131
Hs.730726

3D structure databases

ProteinModelPortaliQ9GZP9
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi119216, 51 interactors
CORUMiQ9GZP9
IntActiQ9GZP9, 34 interactors
MINTiQ9GZP9
STRINGi9606.ENSP00000158771

Protein family/group databases

TCDBi3.A.16.1.4 the endoplasmic reticular retrotranslocon (er-rt) family

PTM databases

iPTMnetiQ9GZP9
PhosphoSitePlusiQ9GZP9

Polymorphism and mutation databases

BioMutaiDERL2
DMDMi50400648

Proteomic databases

EPDiQ9GZP9
MaxQBiQ9GZP9
PaxDbiQ9GZP9
PeptideAtlasiQ9GZP9
PRIDEiQ9GZP9
ProteomicsDBi80110

Protocols and materials databases

DNASUi51009
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000158771; ENSP00000158771; ENSG00000072849
GeneIDi51009
KEGGihsa:51009
UCSCiuc002gcc.2 human

Organism-specific databases

CTDi51009
DisGeNETi51009
EuPathDBiHostDB:ENSG00000072849.10
GeneCardsiDERL2
HGNCiHGNC:17943 DERL2
HPAiCAB037287
HPA048957
HPA056551
MIMi610304 gene
neXtProtiNX_Q9GZP9
OpenTargetsiENSG00000072849
PharmGKBiPA134896343
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0858 Eukaryota
COG5291 LUCA
GeneTreeiENSGT00530000063156
HOGENOMiHOG000200948
HOVERGENiHBG051338
InParanoidiQ9GZP9
KOiK13989
OMAiWWRRLFE
OrthoDBiEOG091G0IPJ
PhylomeDBiQ9GZP9
TreeFamiTF314715

Enzyme and pathway databases

ReactomeiR-HSA-382556 ABC-family proteins mediated transport
R-HSA-5358346 Hedgehog ligand biogenesis
R-HSA-5362768 Hh mutants that don't undergo autocatalytic processing are degraded by ERAD
R-HSA-5678895 Defective CFTR causes cystic fibrosis
R-HSA-901032 ER Quality Control Compartment (ERQC)

Miscellaneous databases

ChiTaRSiDERL2 human
GeneWikiiDerlin-2
GenomeRNAii51009
PROiPR:Q9GZP9
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000072849 Expressed in 220 organ(s), highest expression level in right testis
CleanExiHS_DERL2
ExpressionAtlasiQ9GZP9 baseline and differential
GenevisibleiQ9GZP9 HS

Family and domain databases

InterProiView protein in InterPro
IPR007599 DER1
PANTHERiPTHR11009 PTHR11009, 1 hit
PfamiView protein in Pfam
PF04511 DER1, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiDERL2_HUMAN
AccessioniPrimary (citable) accession number: Q9GZP9
Secondary accession number(s): Q9Y3A7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 19, 2004
Last sequence update: March 1, 2001
Last modified: November 7, 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

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. Human chromosome 17
    Human chromosome 17: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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