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Protein

ER degradation-enhancing alpha-mannosidase-like protein 3

Gene

EDEM3

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

Functioni

Involved in endoplasmic reticulum-associated degradation (ERAD). Accelerates the glycoprotein ERAD by proteasomes, by catalyzing mannose trimming from Man8GlcNAc2 to Man7GlcNAc2 in the N-glycans. Seems to have alpha 1,2-mannosidase activity (By similarity).By similarity1 Publication

Catalytic activityi

Hydrolysis of the terminal (1->2)-linked alpha-D-mannose residues in the oligo-mannose oligosaccharide Man9(GlcNAc)2.By similarity

Cofactori

Ca2+By similarity

Pathwayi: protein glycosylation

This protein is involved in the pathway protein glycosylation, which is part of Protein modification.By similarity
View all proteins of this organism that are known to be involved in the pathway protein glycosylation and in Protein modification.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei146Proton donorBy similarity1
Active sitei293By similarity1
Active sitei387Proton donorBy similarity1
Active sitei405By similarity1
Metal bindingi491CalciumBy similarity1

GO - Molecular functioni

  • calcium ion binding Source: InterPro
  • mannosyl-oligosaccharide 1,2-alpha-mannosidase activity Source: ParkinsonsUK-UCL

GO - Biological processi

Keywordsi

Molecular functionHydrolase
Biological processUnfolded protein response
LigandMetal-binding

Enzyme and pathway databases

ReactomeiR-HSA-901032 ER Quality Control Compartment (ERQC)
UniPathwayiUPA00378

Protein family/group databases

CAZyiGH47 Glycoside Hydrolase Family 47

Names & Taxonomyi

Protein namesi
Recommended name:
ER degradation-enhancing alpha-mannosidase-like protein 3 (EC:3.2.1.113By similarity)
Alternative name(s):
Alpha-1,2-mannosidase EDEM3
Gene namesi
Name:EDEM3
Synonyms:C1orf22
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

EuPathDBiHostDB:ENSG00000116406.18
HGNCiHGNC:16787 EDEM3
MIMi610214 gene
neXtProtiNX_Q9BZQ6

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

Keywords - Cellular componenti

Endoplasmic reticulum

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000116406
PharmGKBiPA38186

Polymorphism and mutation databases

BioMutaiEDEM3
DMDMi166897965

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 41Sequence analysisAdd BLAST41
ChainiPRO_000021032342 – 932ER degradation-enhancing alpha-mannosidase-like protein 3Add BLAST891

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi118N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi195N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi504N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi511N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi810N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi814N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi900N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiQ9BZQ6
MaxQBiQ9BZQ6
PaxDbiQ9BZQ6
PeptideAtlasiQ9BZQ6
PRIDEiQ9BZQ6

PTM databases

iPTMnetiQ9BZQ6
PhosphoSitePlusiQ9BZQ6

Expressioni

Gene expression databases

BgeeiENSG00000116406
CleanExiHS_EDEM3
ExpressionAtlasiQ9BZQ6 baseline and differential
GenevisibleiQ9BZQ6 HS

Organism-specific databases

HPAiHPA025754
HPA025755
HPA027297

Interactioni

Protein-protein interaction databases

BioGridi123207, 66 interactors
IntActiQ9BZQ6, 7 interactors
MINTiQ9BZQ6
STRINGi9606.ENSP00000318147

Structurei

3D structure databases

ProteinModelPortaliQ9BZQ6
SMRiQ9BZQ6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini674 – 779PAAdd BLAST106

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi929 – 932Prevents secretion from ERPROSITE-ProRule annotation4

Domaini

Contains a protease-associated domain (PA) of unknown function.

Sequence similaritiesi

Belongs to the glycosyl hydrolase 47 family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG2429 Eukaryota
ENOG410XS6F LUCA
GeneTreeiENSGT00530000063069
HOGENOMiHOG000007735
HOVERGENiHBG107835
InParanoidiQ9BZQ6
KOiK10086
OMAiCPNTQIL
OrthoDBiEOG091G04YS
PhylomeDBiQ9BZQ6
TreeFamiTF300807

Family and domain databases

CDDicd02126 PA_EDEM3_like, 1 hit
Gene3Di1.50.10.10, 1 hit
InterProiView protein in InterPro
IPR012341 6hp_glycosidase-like_sf
IPR037322 EDEM3_PA
IPR001382 Glyco_hydro_47
IPR003137 PA_domain
IPR036026 Seven-hairpin_glycosidases
PfamiView protein in Pfam
PF01532 Glyco_hydro_47, 1 hit
PF02225 PA, 1 hit
PRINTSiPR00747 GLYHDRLASE47
SUPFAMiSSF48225 SSF48225, 1 hit
PROSITEiView protein in PROSITE
PS00014 ER_TARGET, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9BZQ6-1) [UniParc]FASTAAdd to basket

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.

« Hide

        10         20         30         40         50
MSEAGGRGCG SPVPQRARWR LVAATAAFCL VSATSVWTAG AEPMSREEKQ
60 70 80 90 100
KLGNQVLEMF DHAYGNYMEH AYPADELMPL TCRGRVRGQE PSRGDVDDAL
110 120 130 140 150
GKFSLTLIDS LDTLVVLNKT KEFEDAVRKV LRDVNLDNDV VVSVFETNIR
160 170 180 190 200
VLGGLLGGHS LAIMLKEKGE YMQWYNDELL QMAKQLGYKL LPAFNTTSGL
210 220 230 240 250
PYPRINLKFG IRKPEARTGT ETDTCTACAG TLILEFAALS RFTGATIFEE
260 270 280 290 300
YARKALDFLW EKRQRSSNLV GVTINIHTGD WVRKDSGVGA GIDSYYEYLL
310 320 330 340 350
KAYVLLGDDS FLERFNTHYD AIMRYISQPP LLLDVHIHKP MLNARTWMDA
360 370 380 390 400
LLAFFPGLQV LKGDIRPAIE THEMLYQVIK KHNFLPEAFT TDFRVHWAQH
410 420 430 440 450
PLRPEFAEST YFLYKATGDP YYLEVGKTLI ENLNKYARVP CGFAAMKDVR
460 470 480 490 500
TGSHEDRMDS FFLAEMFKYL YLLFADKEDI IFDIEDYIFT TEAHLLPLWL
510 520 530 540 550
STTNQSISKK NTTSEYTELD DSNFDWTCPN TQILFPNDPL YAQSIREPLK
560 570 580 590 600
NVVDKSCPRG IIRVEESFRS GAKPPLRARD FMATNPEHLE ILKKMGVSLI
610 620 630 640 650
HLKDGRVQLV QHAIQAASSI DAEDGLRFMQ EMIELSSQQQ KEQQLPPRAV
660 670 680 690 700
QIVSHPFFGR VVLTAGPAQF GLDLSKHKET RGFVASSKPS NGCSELTNPE
710 720 730 740 750
AVMGKIALIQ RGQCMFAEKA RNIQNAGAIG GIVIDDNEGS SSDTAPLFQM
760 770 780 790 800
AGDGKDTDDI KIPMLFLFSK EGSIILDAIR EYEEVEVLLS DKAKDRDPEM
810 820 830 840 850
ENEEQPSSEN DSQNQSGEQI SSSSQEVDLV DQESSEENSL NSHPESLSLA
860 870 880 890 900
DMDNAASISP SEQTSNPTEN HETTNLNGEC TDLDNQLQEQ SETEEDSNPN
910 920 930
VSWGKKVQPI DSILADWNED IEAFEMMEKD EL
Length:932
Mass (Da):104,664
Last modified:February 5, 2008 - v2
Checksum:i3C040FDF48284D36
GO
Isoform 2 (identifier: Q9BZQ6-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-43: Missing.
     796-797: RD → RAAILKGKMIPSYIINSN

Note: No experimental confirmation available.
Show »
Length:905
Mass (Da):102,005
Checksum:iA774AE3B184729A4
GO

Sequence cautioni

The sequence AAG60613 differs from that shown. Reason: Erroneous initiation.Curated
The sequence BAG37573 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti276I → T in BAG37573 (PubMed:14702039).Curated1
Sequence conflicti318 – 319HY → VS in AAI05587 (PubMed:11318611).Curated2

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_059306820I → S1 PublicationCorresponds to variant dbSNP:rs9425343Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0563751 – 43Missing in isoform 2. 1 PublicationAdd BLAST43
Alternative sequenceiVSP_056376796 – 797RD → RAAILKGKMIPSYIINSN in isoform 2. 1 Publication2

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK315118 mRNA Translation: BAG37573.1 Different initiation.
AL096819 Genomic DNA No translation available.
BC016464 mRNA Translation: AAH16464.2
BC105586 mRNA Translation: AAI05587.1
BC144149 mRNA Translation: AAI44150.1
AF288393 mRNA Translation: AAG60613.1 Different initiation.
AL117441 mRNA Translation: CAB55926.1
CCDSiCCDS1363.2 [Q9BZQ6-1]
PIRiT17236
RefSeqiNP_001306889.1, NM_001319960.1
NP_079467.3, NM_025191.3 [Q9BZQ6-1]
UniGeneiHs.523811

Genome annotation databases

EnsembliENST00000318130; ENSP00000318147; ENSG00000116406 [Q9BZQ6-1]
ENST00000367512; ENSP00000356482; ENSG00000116406 [Q9BZQ6-2]
GeneIDi80267
KEGGihsa:80267
UCSCiuc010pok.3 human [Q9BZQ6-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiEDEM3_HUMAN
AccessioniPrimary (citable) accession number: Q9BZQ6
Secondary accession number(s): B2RCH6
, B7ZLZ2, Q0VGM5, Q5TEZ0, Q7L2Y5, Q9HCW1, Q9UFV7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 16, 2001
Last sequence update: February 5, 2008
Last modified: March 28, 2018
This is version 147 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

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