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Protein

Derlin-2

Gene

DERL2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

  • endoplasmic reticulum mannose trimming Source: Reactome
  • endoplasmic reticulum unfolded protein response Source: UniProtKB
  • negative regulation of retrograde protein transport, ER to cytosol Source: ParkinsonsUK-UCL
  • positive regulation of cell growth Source: UniProtKB
  • positive regulation of cell proliferation Source: UniProtKB
  • retrograde protein transport, ER to cytosol Source: UniProtKB
  • suckling behavior Source: Ensembl
  • ubiquitin-dependent ERAD pathway Source: UniProtKB

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).

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.

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.

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.
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

Show more details

Protein-protein interaction databases

BioGridi119216. 46 interactors.
CORUMiQ9GZP9.
IntActiQ9GZP9. 32 interactors.
MINTiMINT-3065126.
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.
OMAiGRSPAHF.
OrthoDBiEOG091G0IPJ.
PhylomeDBiQ9GZP9.
TreeFamiTF314715.

Family and domain databases

Gene3Di1.20.1540.10. 1 hit.
InterProiView protein in InterPro
IPR007599. DER1.
IPR035952. Rhomboid-like_sf.
PANTHERiPTHR11009. PTHR11009. 1 hit.
SUPFAMiSSF144091. SSF144091. 1 hit.

Sequencei

Sequence statusi: Complete.

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

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

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: October 25, 2017
This is version 137 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. Human chromosome 17
    Human chromosome 17: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families