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Q96Q80 (DERL3_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 98. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Derlin-3
Alternative name(s):
Degradation in endoplasmic reticulum protein 3
Short name=DERtrin-3
Der1-like protein 3
Gene names
Name:DERL3
Synonyms:C22orf14, DER3, LLN2
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length235 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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 the degradation substrate. Ref.7 Ref.9

Subunit structure

Forms homo- and heterooligomers with DERL2 and, to a lesser extent, with DERL1. Interacts with VCP, EDEM1, SELK and VIMP/SELS. Ref.7 Ref.8

Subcellular location

Endoplasmic reticulum membrane; Multi-pass membrane protein Ref.7.

Tissue specificity

Unlike DERL1 and DERL2, restricted to several tissues. Expressed at high levels in placenta, pancreas, spleen and small intestine. Ref.7

Induction

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

Sequence similarities

Belongs to the derlin family.

Alternative products

This entry describes 5 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q96Q80-1)

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.
Isoform 2 (identifier: Q96Q80-2)

The sequence of this isoform differs from the canonical sequence as follows:
     206-235: KLLLDAPAEDPNYLPLPEEQPGPHLPPPQQ → GLQSSKAPAGSSLTIWTQQSQGGPGTAGELAAPS
Note: No experimental confirmation available.
Isoform 3 (identifier: Q96Q80-3)

The sequence of this isoform differs from the canonical sequence as follows:
     206-235: KLLLDAPAEDPNYLPLPEEQPGPHLPPPQQ → LATAQQCPHRTGPSAGDFRAARPQLAVA
Note: No experimental confirmation available.
Isoform 4 (identifier: Q96Q80-4)

The sequence of this isoform differs from the canonical sequence as follows:
     1-74: Missing.
     110-205: LLGLLGSLFF...KRLLQTPGFL → VSFPQALEPR...LCSTRAPPHR
Note: No experimental confirmation available.
Isoform 5 (identifier: Q96Q80-5)

The sequence of this isoform differs from the canonical sequence as follows:
     206-235: Missing.
Note: No experimental confirmation available.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 235235Derlin-3
PRO_0000219048

Regions

Topological domain1 – 2222Cytoplasmic Potential
Transmembrane23 – 4321Helical; Name=1; Potential
Topological domain44 – 5815Lumenal Potential
Transmembrane59 – 7921Helical; Name=2; Potential
Topological domain80 – 9819Cytoplasmic Potential
Transmembrane99 – 11921Helical; Name=3; Potential
Topological domain120 – 15738Lumenal Potential
Transmembrane158 – 17821Helical; Name=4; Potential
Topological domain179 – 23557Cytoplasmic Potential

Natural variations

Alternative sequence1 – 7474Missing in isoform 4.
VSP_011086
Alternative sequence110 – 20596LLGLL…TPGFL → VSFPQALEPRARAPRRPACV GPGANTAMPERDTVAVSSLV CVEGPLCAQLQGSGLDLQCC MQNTKPRTKEPGTVPALGAH GLLAAAGQLHPRGPAGDCGG PYLLLPGGRLPQPAWRQEAP ADPWLPVSVESPPSLSPPSE GSPPMGTCAGLCSTRAPPHR in isoform 4.
VSP_011087
Alternative sequence206 – 23530KLLLD…PPPQQ → GLQSSKAPAGSSLTIWTQQS QGGPGTAGELAAPS in isoform 2.
VSP_011088
Alternative sequence206 – 23530KLLLD…PPPQQ → LATAQQCPHRTGPSAGDFRA ARPQLAVA in isoform 3.
VSP_011089
Alternative sequence206 – 23530Missing in isoform 5.
VSP_046330
Natural variant1491F → L. Ref.2
Corresponds to variant rs3177243 [ dbSNP | Ensembl ].
VAR_048897
Natural variant2111A → V. Ref.3
Corresponds to variant rs1128127 [ dbSNP | Ensembl ].
VAR_019517

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified July 19, 2004. Version 2.
Checksum: 2DC5F02487C4B824

FASTA23526,679
        10         20         30         40         50         60 
MAWQGLAAEF LQVPAVTRAY TAACVLTTAA VQLELLSPFQ LYFNPHLVFR KFQVWRLVTN 

        70         80         90        100        110        120 
FLFFGPLGFS FFFNMLFVFR YCRMLEEGSF RGRTADFVFM FLFGGVLMTL LGLLGSLFFL 

       130        140        150        160        170        180 
GQALMAMLVY VWSRRSPRVR VNFFGLLTFQ APFLPWALMG FSLLLGNSIL VDLLGIAVGH 

       190        200        210        220        230 
IYYFLEDVFP NQPGGKRLLQ TPGFLKLLLD APAEDPNYLP LPEEQPGPHL PPPQQ 

« Hide

Isoform 2 [UniParc].

Checksum: 7297772E29193957
Show »

FASTA23926,605
Isoform 3 [UniParc].

Checksum: 98A860411ED2C73B
Show »

FASTA23326,255
Isoform 4 [UniParc].

Checksum: 30576E135EC94D48
Show »

FASTA22523,859
Isoform 5 [UniParc].

Checksum: E125D7D2EDBB8031
Show »

FASTA20523,382

References

« Hide 'large scale' references
[1]"Molecular cloning of a novel member of the NADH oxidoreductase complex I subunit homolog."
Shimizu N., Minosima S., Kawasaki K., Sasaki T.
Submitted (SEP-2000) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE (ISOFORM 3).
[2]The European IMAGE consortium
Submitted (JUL-2000) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 5), VARIANT LEU-149.
[3]"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. expand/collapse author list , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 4), VARIANT VAL-211.
Tissue: Testis.
[4]"A genome annotation-driven approach to cloning the human ORFeome."
Collins J.E., Wright C.L., Edwards C.A., Davis M.P., Grinham J.A., Cole C.G., Goward M.E., Aguado B., Mallya M., Mokrab Y., Huckle E.J., Beare D.M., Dunham I.
Genome Biol. 5:R84.1-R84.11(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
[5]"The DNA sequence of human chromosome 22."
Dunham I., Hunt A.R., Collins J.E., Bruskiewich R., Beare D.M., Clamp M., Smink L.J., Ainscough R., Almeida J.P., Babbage A.K., Bagguley C., Bailey J., Barlow K.F., Bates K.N., Beasley O.P., Bird C.P., Blakey S.E., Bridgeman A.M. expand/collapse author list , Buck D., Burgess J., Burrill W.D., Burton J., Carder C., Carter N.P., Chen Y., Clark G., Clegg S.M., Cobley V.E., Cole C.G., Collier R.E., Connor R., Conroy D., Corby N.R., Coville G.J., Cox A.V., Davis J., Dawson E., Dhami P.D., Dockree C., Dodsworth S.J., Durbin R.M., Ellington A.G., Evans K.L., Fey J.M., Fleming K., French L., Garner A.A., Gilbert J.G.R., Goward M.E., Grafham D.V., Griffiths M.N.D., Hall C., Hall R.E., Hall-Tamlyn G., Heathcott R.W., Ho S., Holmes S., Hunt S.E., Jones M.C., Kershaw J., Kimberley A.M., King A., Laird G.K., Langford C.F., Leversha M.A., Lloyd C., Lloyd D.M., Martyn I.D., Mashreghi-Mohammadi M., Matthews L.H., Mccann O.T., Mcclay J., Mclaren S., McMurray A.A., Milne S.A., Mortimore B.J., Odell C.N., Pavitt R., Pearce A.V., Pearson D., Phillimore B.J.C.T., Phillips S.H., Plumb R.W., Ramsay H., Ramsey Y., Rogers L., Ross M.T., Scott C.E., Sehra H.K., Skuce C.D., Smalley S., Smith M.L., Soderlund C., Spragon L., Steward C.A., Sulston J.E., Swann R.M., Vaudin M., Wall M., Wallis J.M., Whiteley M.N., Willey D.L., Williams L., Williams S.A., Williamson H., Wilmer T.E., Wilming L., Wright C.L., Hubbard T., Bentley D.R., Beck S., Rogers J., Shimizu N., Minoshima S., Kawasaki K., Sasaki T., Asakawa S., Kudoh J., Shintani A., Shibuya K., Yoshizaki Y., Aoki N., Mitsuyama S., Roe B.A., Chen F., Chu L., Crabtree J., Deschamps S., Do A., Do T., Dorman A., Fang F., Fu Y., Hu P., Hua A., Kenton S., Lai H., Lao H.I., Lewis J., Lewis S., Lin S.-P., Loh P., Malaj E., Nguyen T., Pan H., Phan S., Qi S., Qian Y., Ray L., Ren Q., Shaull S., Sloan D., Song L., Wang Q., Wang Y., Wang Z., White J., Willingham D., Wu H., Yao Z., Zhan M., Zhang G., Chissoe S., Murray J., Miller N., Minx P., Fulton R., Johnson D., Bemis G., Bentley D., Bradshaw H., Bourne S., Cordes M., Du Z., Fulton L., Goela D., Graves T., Hawkins J., Hinds K., Kemp K., Latreille P., Layman D., Ozersky P., Rohlfing T., Scheet P., Walker C., Wamsley A., Wohldmann P., Pepin K., Nelson J., Korf I., Bedell J.A., Hillier L.W., Mardis E., Waterston R., Wilson R., Emanuel B.S., Shaikh T., Kurahashi H., Saitta S., Budarf M.L., McDermid H.E., Johnson A., Wong A.C.C., Morrow B.E., Edelmann L., Kim U.J., Shizuya H., Simon M.I., Dumanski J.P., Peyrard M., Kedra D., Seroussi E., Fransson I., Tapia I., Bruder C.E., O'Brien K.P., Wilkinson P., Bodenteich A., Hartman K., Hu X., Khan A.S., Lane L., Tilahun Y., Wright H.
Nature 402:489-495(1999) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[6]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
Tissue: Placenta.
[7]"Derlin-2 and Derlin-3 are regulated by the mammalian unfolded protein response and are required for ER-associated degradation."
Oda Y., Okada T., Yoshida H., Kaufman R.J., Nagata K., Mori K.
J. Cell Biol. 172:383-393(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, TISSUE SPECIFICITY, SUBCELLULAR LOCATION, MEMBRANE TOPOLOGY, OLIGOMERIZATION, INTERACTION WITH VCP AND EDEM1.
[8]"Selenoprotein K binds multiprotein complexes and is involved in the regulation of endoplasmic reticulum homeostasis."
Shchedrina V.A., Everley R.A., Zhang Y., Gygi S.P., Hatfield D.L., Gladyshev V.N.
J. Biol. Chem. 286:42937-42948(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: INTERACTION WITH SELK AND VIMP.
[9]"STT3B-dependent posttranslational N-glycosylation as a surveillance system for secretory protein."
Sato T., Sako Y., Sho M., Momohara M., Suico M.A., Shuto T., Nishitoh H., Okiyoneda T., Kokame K., Kaneko M., Taura M., Miyata M., Chosa K., Koga T., Morino-Koga S., Wada I., Kai H.
Mol. Cell 47:99-110(2012) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION IN ERAD PATHWAY.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AB049213 Genomic DNA. Translation: BAB68409.1.
AL389876 mRNA. No translation available.
AK125830 mRNA. Translation: BAC86311.1.
CR456372 mRNA. Translation: CAG30258.1.
AP000350 Genomic DNA. No translation available.
BC057830 mRNA. Translation: AAH57830.1.
CCDSCCDS33615.1. [Q96Q80-1]
CCDS42986.1. [Q96Q80-5]
CCDS46672.1. [Q96Q80-2]
RefSeqNP_001002862.1. NM_001002862.2. [Q96Q80-1]
NP_001129223.1. NM_001135751.1. [Q96Q80-2]
NP_940842.2. NM_198440.3. [Q96Q80-5]
UniGeneHs.593679.

3D structure databases

ProteinModelPortalQ96Q80.
SMRQ96Q80. Positions 11-185.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid124816. 3 interactions.
STRING9606.ENSP00000384744.

Polymorphism databases

DMDM50400613.

Proteomic databases

PaxDbQ96Q80.
PRIDEQ96Q80.

Protocols and materials databases

DNASU91319.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000318109; ENSP00000315303; ENSG00000099958. [Q96Q80-1]
ENST00000406855; ENSP00000384744; ENSG00000099958. [Q96Q80-2]
ENST00000476077; ENSP00000419399; ENSG00000099958. [Q96Q80-5]
GeneID91319.
KEGGhsa:91319.
UCSCuc002zyh.3. human. [Q96Q80-1]
uc002zyj.3. human. [Q96Q80-4]
uc002zyk.4. human. [Q96Q80-2]

Organism-specific databases

CTD91319.
GeneCardsGC22M024176.
HGNCHGNC:14236. DERL3.
HPACAB037234.
HPA049306.
MIM610305. gene.
neXtProtNX_Q96Q80.
PharmGKBPA25883.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG5291.
HOGENOMHOG000200948.
HOVERGENHBG051338.
KOK13989.
OrthoDBEOG73RBC2.
PhylomeDBQ96Q80.
TreeFamTF314715.

Gene expression databases

ArrayExpressQ96Q80.
BgeeQ96Q80.
CleanExHS_DERL3.
GenevestigatorQ96Q80.

Family and domain databases

InterProIPR007599. DER1.
[Graphical view]
PANTHERPTHR11009. PTHR11009. 1 hit.
PfamPF04511. DER1. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

GeneWikiDerlin-3.
GenomeRNAi91319.
NextBio77178.
PROQ96Q80.
SOURCESearch...

Entry information

Entry nameDERL3_HUMAN
AccessionPrimary (citable) accession number: Q96Q80
Secondary accession number(s): F2Z3B6 expand/collapse secondary AC list , Q6ICJ6, Q6PEX0, Q6ZUB5
Entry history
Integrated into UniProtKB/Swiss-Prot: July 19, 2004
Last sequence update: July 19, 2004
Last modified: July 9, 2014
This is version 98 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant 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 polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 22

Human chromosome 22: entries, gene names and cross-references to MIM