DERL2

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
Inferred from direct assayⁱ
- 16449189
negative regulation of retrograde protein transport, ER to cytosol
Source: ParkinsonsUK-UCL
Inferred from mutant phenotypeⁱ
- 25660456
positive regulation of cell growth
Source: UniProtKB
Inferred from direct assayⁱ
- 11500051
positive regulation of cell proliferation
Source: UniProtKB
Inferred from direct assayⁱ
- 11500051
retrograde protein transport, ER to cytosol
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 16449189
suckling behavior Source: Ensembl
ubiquitin-dependent ERAD pathway
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 16449189

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Biological process	Unfolded protein response

Biological process

Unfolded protein response

Enzyme and pathway databases

Reactomeⁱ	R-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)

Reactomeⁱ

R-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

TCDBⁱ	3.A.16.1.4 the endoplasmic reticular retrotranslocon (er-rt) family

TCDBⁱ

3.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
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 17

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000072849.10
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:17943 DERL2
MIMⁱ	610304 gene
neXtProtⁱ	NX_Q9GZP9

Topology

Feature key	Position(s)	DescriptionActions	Length
Topological domainⁱ	1 – 57	CytoplasmicSequence analysisAdd BLAST	57
Transmembraneⁱ	58 – 78	Helical; Name=1Sequence analysisAdd BLAST	21
Topological domainⁱ	79 – 96	LumenalSequence analysisAdd BLAST	18
Transmembraneⁱ	97 – 117	Helical; Name=2Sequence analysisAdd BLAST	21
Topological domainⁱ	118 – 150	CytoplasmicSequence analysisAdd BLAST	33
Transmembraneⁱ	151 – 171	Helical; Name=3Sequence analysisAdd BLAST	21
Topological domainⁱ	172	LumenalSequence analysis	1
Transmembraneⁱ	173 – 193	Helical; Name=4Sequence analysisAdd BLAST	21
Topological domainⁱ	194 – 239	CytoplasmicSequence analysisAdd BLAST	46

Keywords - Cellular componentⁱ

Endoplasmic reticulum, Membrane

Pathology & Biotechⁱ

Organism-specific databases

DisGeNET More...DisGeNETⁱ	51009
Open Targets More...OpenTargetsⁱ	ENSG00000072849
PharmGKBⁱ	PA134896343

Polymorphism and mutation databases

BioMutaⁱ	DERL2
DMDMⁱ	50400648

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000219045	1 – 239	Derlin-2Add BLAST		239

Proteomic databases

EPDⁱ	Q9GZP9
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	Q9GZP9
PaxDbⁱ	Q9GZP9
PeptideAtlas More...PeptideAtlasⁱ	Q9GZP9
PRIDEⁱ	Q9GZP9
ProteomicsDBⁱ	80110

PTM databases

iPTMnetⁱ	Q9GZP9
PhosphoSitePlusⁱ	Q9GZP9

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

Bgeeⁱ	ENSG00000072849 Expressed in 220 organ(s), highest expression level in right testis
CleanExⁱ	HS_DERL2
ExpressionAtlasⁱ	Q9GZP9 baseline and differential
Genevisibleⁱ	Q9GZP9 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	CAB037287 HPA048957 HPA056551

HPAⁱ

CAB037287
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

With	Entry	#Exp.	IntAct	Notes
KRTAP10-7	P60409	3	EBI-7962814,EBI-10172290
KRTAP10-8	P60410	3	EBI-7962814,EBI-10171774

Protein-protein interaction databases

BioGridⁱ	119216, 51 interactors
CORUMⁱ	Q9GZP9
IntActⁱ	Q9GZP9, 34 interactors
Molecular INTeraction database More...MINTⁱ	Q9GZP9
STRINGⁱ	9606.ENSP00000158771

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q9GZP9
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Sequence similaritiesⁱ

Belongs to the derlin family.Curated

Keywords - Domainⁱ

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGⁱ	KOG0858 Eukaryota COG5291 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00530000063156
HOGENOMⁱ	HOG000200948
HOVERGENⁱ	HBG051338
InParanoidⁱ	Q9GZP9
KEGG Orthology (KO) More...KOⁱ	K13989
OMAⁱ	WWRRLFE
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0IPJ
PhylomeDBⁱ	Q9GZP9
TreeFamⁱ	TF314715

Family and domain databases

InterProⁱ	View protein in InterPro IPR007599 DER1
PANTHERⁱ	PTHR11009 PTHR11009, 1 hit
Pfam protein domain database More...Pfamⁱ	View 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 all Align All

Q9GZP9-1 [UniParc]FASTA Add 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.BLAST Align Show all Add to basket

Entry	Entry name	Protein names	Gene names	Length	Annotation

I3L1S8	I3L1S8_HUMAN	Derlin Derlin	DERL2	144	Annotation score:
I3L1T3	I3L1T3_HUMAN	Derlin Derlin	DERL2	158	Annotation score:
I3L0R8	I3L0R8_HUMAN	Derlin Derlin	DERL2	200	Annotation score:
I3L1W3	I3L1W3_HUMAN	Derlin Derlin	DERL2	103	Annotation score:
I3L4W7	I3L4W7_HUMAN	Derlin-2 Derlin-2	DERL2	79	Annotation score:
K7EQE8	K7EQE8_HUMAN	Derlin-2 Derlin-2	DERL2	82	Annotation score:
I3L3R8	I3L3R8_HUMAN	Derlin-2 Derlin-2	DERL2	74	Annotation 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
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS11073.1
PIRⁱ	JC7752
NCBI Reference Sequences More...RefSeqⁱ	NP_001291708.1, NM_001304779.1 NP_057125.2, NM_016041.4
UniGeneⁱ	Hs.286131 Hs.730726

Genome annotation databases

Ensemblⁱ	ENST00000158771; ENSP00000158771; ENSG00000072849
GeneIDⁱ	51009
KEGGⁱ	hsa:51009
UCSC genome browser More...UCSCⁱ	uc002gcc.2 human

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
Q9GZP9	Derlin-2		PONAB		239	UniRef100_Q9GZP9
Derlin		CALJA		239
Derlin		PANTR		239
Derlin		MACMU		239
Derlin (Fragment)		AILME		239
+29

Protein	Similar proteins		Species	Score	Length	Source
Q9GZP9	Derlin-2		PONAB		239	UniRef90_Q9GZP9
Derlin		CALJA		239
Derlin		PANTR		239
Derlin		MACMU		239
Derlin (Fragment)		AILME		239
+73

Protein	Similar proteins		Species	Score	Length	Source
Q9GZP9	Derlin-2		PONAB		239	UniRef50_Q9GZP9
Derlin-3		HUMAN		235
Derlin-3		BOVIN		231
Derlin		CALJA		239
Derlin		PANTR		239
+360

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
CCDSⁱ	CCDS11073.1
PIRⁱ	JC7752
RefSeqⁱ	NP_001291708.1, NM_001304779.1 NP_057125.2, NM_016041.4
UniGeneⁱ	Hs.286131 Hs.730726

3D structure databases

ProteinModelPortalⁱ	Q9GZP9
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	119216, 51 interactors
CORUMⁱ	Q9GZP9
IntActⁱ	Q9GZP9, 34 interactors
MINTⁱ	Q9GZP9
STRINGⁱ	9606.ENSP00000158771

Protein family/group databases

TCDBⁱ	3.A.16.1.4 the endoplasmic reticular retrotranslocon (er-rt) family

TCDBⁱ

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

PTM databases

iPTMnetⁱ	Q9GZP9
PhosphoSitePlusⁱ	Q9GZP9

Polymorphism and mutation databases

BioMutaⁱ	DERL2
DMDMⁱ	50400648

Proteomic databases

EPDⁱ	Q9GZP9
MaxQBⁱ	Q9GZP9
PaxDbⁱ	Q9GZP9
PeptideAtlasⁱ	Q9GZP9
PRIDEⁱ	Q9GZP9
ProteomicsDBⁱ	80110

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	51009
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000158771; ENSP00000158771; ENSG00000072849
GeneIDⁱ	51009
KEGGⁱ	hsa:51009
UCSCⁱ	uc002gcc.2 human

Organism-specific databases

CTDⁱ	51009
DisGeNETⁱ	51009
EuPathDBⁱ	HostDB:ENSG00000072849.10
GeneCardsⁱ	DERL2
HGNCⁱ	HGNC:17943 DERL2
HPAⁱ	CAB037287 HPA048957 HPA056551
MIMⁱ	610304 gene
neXtProtⁱ	NX_Q9GZP9
OpenTargetsⁱ	ENSG00000072849
PharmGKBⁱ	PA134896343
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG0858 Eukaryota COG5291 LUCA
GeneTreeⁱ	ENSGT00530000063156
HOGENOMⁱ	HOG000200948
HOVERGENⁱ	HBG051338
InParanoidⁱ	Q9GZP9
KOⁱ	K13989
OMAⁱ	WWRRLFE
OrthoDBⁱ	EOG091G0IPJ
PhylomeDBⁱ	Q9GZP9
TreeFamⁱ	TF314715

Enzyme and pathway databases

Reactomeⁱ	R-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)

Reactomeⁱ

R-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

ChiTaRSⁱ	DERL2 human
GeneWikiⁱ	Derlin-2
GenomeRNAiⁱ	51009
Protein Ontology More...PROⁱ	PR:Q9GZP9
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000072849 Expressed in 220 organ(s), highest expression level in right testis
CleanExⁱ	HS_DERL2
ExpressionAtlasⁱ	Q9GZP9 baseline and differential
Genevisibleⁱ	Q9GZP9 HS

Family and domain databases

InterProⁱ	View protein in InterPro IPR007599 DER1
PANTHERⁱ	PTHR11009 PTHR11009, 1 hit
Pfamⁱ	View protein in Pfam PF04511 DER1, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	DERL2_HUMAN
Accessionⁱ	Q9GZP9Primary (citable) accession number: Q9GZP9 Secondary accession number(s): Q9Y3A7
Entry historyⁱ	Integrated 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 statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any 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

SIMILARITY comments
Index of protein domains and families
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
Human chromosome 17
Human chromosome 17: entries, gene names and cross-references to MIM

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UniProtKB - Q9GZP9 (DERL2_HUMAN)

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Derlin-2

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Biological processi

Enzyme and pathway databases

Protein family/group databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Endoplasmic reticulum

Endoplasmic reticulum

Endosome

Other locations

Topology

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Organism-specific databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Keywords - Domaini

Phylogenomic databases

Family and domain databases

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

Sequence databases

Genome annotation databases

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

Protein family/group databases

PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Documents

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Functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Sequence cautionⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ