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Protein

Translocation protein SEC63 homolog

Gene

SEC63

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

Functioni

Required for integral membrane and secreted preprotein translocation across the endoplasmic reticulum membrane.

GO - Molecular functioni

  • protein transporter activity Source: InterPro
  • receptor activity Source: ProtInc
  • RNA binding Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionChaperone
Biological processProtein transport, Transport

Enzyme and pathway databases

ReactomeiR-HSA-381038. XBP1(S) activates chaperone genes.

Protein family/group databases

TCDBi3.A.5.9.1. the general secretory pathway (sec) family.

Names & Taxonomyi

Protein namesi
Recommended name:
Translocation protein SEC63 homolog
Gene namesi
Name:SEC63
Synonyms:SEC63L
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

HGNCiHGNC:21082. SEC63.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 14LumenalSequence analysisAdd BLAST14
Transmembranei15 – 35HelicalSequence analysisAdd BLAST21
Topological domaini36 – 69CytoplasmicSequence analysisAdd BLAST34
Transmembranei70 – 90HelicalSequence analysisAdd BLAST21
Topological domaini91 – 188LumenalSequence analysisAdd BLAST98
Transmembranei189 – 209HelicalSequence analysisAdd BLAST21
Topological domaini210 – 760CytoplasmicSequence analysisAdd BLAST551

GO - Cellular componenti

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane

Pathology & Biotechi

Involvement in diseasei

Polycystic liver disease 2 (PCLD2)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal dominant hepatobiliary disease characterized by overgrowth of biliary epithelium and supportive connective tissue, resulting in multiple liver cysts.
See also OMIM:617004
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_019645568Missing in PCLD2. 1 Publication1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi11231.
MalaCardsiSEC63.
MIMi617004. phenotype.
OpenTargetsiENSG00000025796.
Orphaneti2924. Isolated polycystic liver disease.
PharmGKBiPA134936990.

Polymorphism and mutation databases

BioMutaiSEC63.
DMDMi18203500.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000710971 – 760Translocation protein SEC63 homologAdd BLAST760

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei537PhosphothreonineCombined sources1
Modified residuei742PhosphoserineCombined sources1
Modified residuei748PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ9UGP8.
MaxQBiQ9UGP8.
PaxDbiQ9UGP8.
PeptideAtlasiQ9UGP8.
PRIDEiQ9UGP8.

PTM databases

iPTMnetiQ9UGP8.
PhosphoSitePlusiQ9UGP8.
SwissPalmiQ9UGP8.

Expressioni

Tissue specificityi

Widely expressed, with high levels in the liver.1 Publication

Gene expression databases

BgeeiENSG00000025796.
CleanExiHS_SEC63.
ExpressionAtlasiQ9UGP8. baseline and differential.
GenevisibleiQ9UGP8. HS.

Organism-specific databases

HPAiHPA053295.
HPA060535.

Interactioni

Subunit structurei

Part of the multicomponent translocon that contains the trimeric SEC61 complex, SEC62 and SEC63.

Binary interactionsi

WithEntry#Exp.IntActNotes
NxnP973466EBI-1045560,EBI-309684From Mus musculus.

Protein-protein interaction databases

BioGridi116397. 42 interactors.
IntActiQ9UGP8. 24 interactors.
MINTiMINT-3079991.
STRINGi9606.ENSP00000357998.

Structurei

3D structure databases

ProteinModelPortaliQ9UGP8.
SMRiQ9UGP8.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini104 – 165JPROSITE-ProRule annotationAdd BLAST62
Domaini197 – 541SEC63 1Add BLAST345
Domaini637 – 714SEC63 2Add BLAST78

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili597 – 635Sequence analysisAdd BLAST39

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi734 – 760Asp/Glu-rich (highly acidic)Add BLAST27

Keywords - Domaini

Coiled coil, Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0721. Eukaryota.
KOG4434. Eukaryota.
COG5407. LUCA.
GeneTreeiENSGT00390000001965.
HOGENOMiHOG000037933.
HOVERGENiHBG057075.
InParanoidiQ9UGP8.
KOiK09540.
OMAiSQFDYEM.
OrthoDBiEOG091G03YI.
PhylomeDBiQ9UGP8.
TreeFamiTF105904.

Family and domain databases

CDDicd06257. DnaJ. 1 hit.
Gene3Di1.10.287.110. 1 hit.
2.60.40.150. 1 hit.
InterProiView protein in InterPro
IPR000008. C2_dom.
IPR001623. DnaJ_domain.
IPR014756. Ig_E-set.
IPR027137. Sec63.
IPR004179. Sec63-dom.
PANTHERiPTHR24075:SF5. PTHR24075:SF5. 1 hit.
PfamiView protein in Pfam
PF00226. DnaJ. 1 hit.
PF02889. Sec63. 2 hits.
PRINTSiPR00625. JDOMAIN.
SMARTiView protein in SMART
SM00271. DnaJ. 1 hit.
SM00973. Sec63. 1 hit.
SUPFAMiSSF46565. SSF46565. 1 hit.
SSF81296. SSF81296. 2 hits.
PROSITEiView protein in PROSITE
PS50076. DNAJ_2. 1 hit.

Sequencei

Sequence statusi: Complete.

Q9UGP8-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGQQFQYDD SGNTFFYFLT SFVGLIVIPA TYYLWPRDQN AEQIRLKNIR
60 70 80 90 100
KVYGRCMWYR LRLLKPQPNI IPTVKKIVLL AGWALFLFLA YKVSKTDREY
110 120 130 140 150
QEYNPYEVLN LDPGATVAEI KKQYRLLSLK YHPDKGGDEV MFMRIAKAYA
160 170 180 190 200
ALTDEESRKN WEEFGNPDGP QATSFGIALP AWIVDQKNSI LVLLVYGLAF
210 220 230 240 250
MVILPVVVGS WWYRSIRYSG DQILIRTTQI YTYFVYKTRN MDMKRLIMVL
260 270 280 290 300
AGASEFDPQY NKDATSRPTD NILIPQLIRE IGSINLKKNE PPLTCPYSLK
310 320 330 340 350
ARVLLLSHLA RMKIPETLEE DQQFMLKKCP ALLQEMVNVI CQLIVMARNR
360 370 380 390 400
EEREFRAPTL ASLENCMKLS QMAVQGLQQF KSPLLQLPHI EEDNLRRVSN
410 420 430 440 450
HKKYKIKTIQ DLVSLKESDR HTLLHFLEDE KYEEVMAVLG SFPYVTMDIK
460 470 480 490 500
SQVLDDEDSN NITVGSLVTV LVKLTRQTMA EVFEKEQSIC AAEEQPAEDG
510 520 530 540 550
QGETNKNRTK GGWQQKSKGP KKTAKSKKKK PLKKKPTPVL LPQSKQQKQK
560 570 580 590 600
QANGVVGNEA AVKEDEEEVS DKGSDSEEEE TNRDSQSEKD DGSDRDSDRE
610 620 630 640 650
QDEKQNKDDE AEWQELQQSI QRKERALLET KSKITHPVYS LYFPEEKQEW
660 670 680 690 700
WWLYIADRKE QTLISMPYHV CTLKDTEEVE LKFPAPGKPG NYQYTVFLRS
710 720 730 740 750
DSYMGLDQIK PLKLEVHEAK PVPENHPQWD TAIEGDEDQE DSEGFEDSFE
760
EEEEEEEDDD
Length:760
Mass (Da):87,997
Last modified:January 23, 2007 - v2
Checksum:i81BBF269A3FCCF0D
GO

Sequence cautioni

The sequence AAH23598 differs from that shown. Contaminating sequence. Potential poly-A sequence.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti115A → V in AAH23598 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_061146556V → I1 PublicationCorresponds to variant dbSNP:rs17854547Ensembl.1
Natural variantiVAR_019645568Missing in PCLD2. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ011779 mRNA. Translation: CAB46275.1.
AF100141 mRNA. Translation: AAC83375.1.
AL024507 Genomic DNA. No translation available.
BC023598 mRNA. Translation: AAH23598.1. Sequence problems.
BC047221 mRNA. Translation: AAH47221.1.
BC048287 mRNA. Translation: AAH48287.1.
AL137338 mRNA. Translation: CAB70701.1.
CCDSiCCDS5061.1.
PIRiT46504.
RefSeqiNP_009145.1. NM_007214.4.
UniGeneiHs.26904.

Genome annotation databases

EnsembliENST00000369002; ENSP00000357998; ENSG00000025796.
GeneIDi11231.
KEGGihsa:11231.
UCSCiuc003psc.5. human.

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiSEC63_HUMAN
AccessioniPrimary (citable) accession number: Q9UGP8
Secondary accession number(s): O95380
, Q5THN4, Q86VS9, Q8IWL0, Q9NTE0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 18, 2001
Last sequence update: January 23, 2007
Last modified: August 30, 2017
This is version 157 of the entry and version 2 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 6
    Human chromosome 6: 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. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot