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Protein

ER degradation-enhancing alpha-mannosidase-like protein 3

Gene

EDEM3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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)LengthDescriptionGraphical viewFeature identifierActions
Active sitei146 – 1461Proton donorBy similarity
Active sitei293 – 2931By similarity
Active sitei387 – 3871Proton donorBy similarity
Active sitei405 – 4051By similarity
Metal bindingi491 – 4911CalciumBy similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Keywords - Biological processi

Unfolded protein response

Keywords - Ligandi

Metal-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

HGNCiHGNC:16787. EDEM3.

Subcellular locationi

  • Endoplasmic reticulum lumen PROSITE-ProRule annotation

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA38186.

Polymorphism and mutation databases

BioMutaiEDEM3.
DMDMi166897965.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 4141Sequence analysisAdd
BLAST
Chaini42 – 932891ER degradation-enhancing alpha-mannosidase-like protein 3PRO_0000210323Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi118 – 1181N-linked (GlcNAc...)Sequence analysis
Glycosylationi195 – 1951N-linked (GlcNAc...)1 Publication
Glycosylationi504 – 5041N-linked (GlcNAc...)Sequence analysis
Glycosylationi511 – 5111N-linked (GlcNAc...)Sequence analysis
Glycosylationi810 – 8101N-linked (GlcNAc...)Sequence analysis
Glycosylationi814 – 8141N-linked (GlcNAc...)Sequence analysis
Glycosylationi900 – 9001N-linked (GlcNAc...)Sequence analysis

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiQ9BZQ6.
MaxQBiQ9BZQ6.
PaxDbiQ9BZQ6.
PeptideAtlasiQ9BZQ6.
PRIDEiQ9BZQ6.

PTM databases

iPTMnetiQ9BZQ6.
PhosphoSiteiQ9BZQ6.

Expressioni

Gene expression databases

BgeeiENSG00000116406.
CleanExiHS_EDEM3.
ExpressionAtlasiQ9BZQ6. baseline and differential.
GenevisibleiQ9BZQ6. HS.

Organism-specific databases

HPAiHPA025754.
HPA025755.

Interactioni

Protein-protein interaction databases

BioGridi123207. 11 interactions.
IntActiQ9BZQ6. 4 interactions.
STRINGi9606.ENSP00000318147.

Structurei

3D structure databases

ProteinModelPortaliQ9BZQ6.
SMRiQ9BZQ6. Positions 56-499.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini674 – 779106PAAdd
BLAST

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi929 – 9324Prevents secretion from ERPROSITE-ProRule annotation

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.
OMAiMASNPEH.
OrthoDBiEOG091G04YS.
PhylomeDBiQ9BZQ6.
TreeFamiTF300807.

Family and domain databases

Gene3Di1.50.10.50. 1 hit.
InterProiIPR001382. Glyco_hydro_47.
IPR003137. PA_domain.
[Graphical view]
PfamiPF01532. Glyco_hydro_47. 1 hit.
PF02225. PA. 1 hit.
[Graphical view]
PRINTSiPR00747. GLYHDRLASE47.
SUPFAMiSSF48225. SSF48225. 1 hit.
PROSITEiPS00014. ER_TARGET. 1 hit.
[Graphical view]

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)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti276 – 2761I → T in BAG37573 (PubMed:14702039).Curated
Sequence conflicti318 – 3192HY → VS in AAI05587 (PubMed:11318611).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti820 – 8201I → S.1 Publication
Corresponds to variant rs9425343 [ dbSNP | Ensembl ].
VAR_059306

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 4343Missing in isoform 2. 1 PublicationVSP_056375Add
BLAST
Alternative sequencei796 – 7972RD → RAAILKGKMIPSYIINSN in isoform 2. 1 PublicationVSP_056376

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK315118 mRNA. Translation: BAG37573.1. Different initiation.
AL096819 Genomic DNA. Translation: CAI21741.1.
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

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK315118 mRNA. Translation: BAG37573.1. Different initiation.
AL096819 Genomic DNA. Translation: CAI21741.1.
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.

3D structure databases

ProteinModelPortaliQ9BZQ6.
SMRiQ9BZQ6. Positions 56-499.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi123207. 11 interactions.
IntActiQ9BZQ6. 4 interactions.
STRINGi9606.ENSP00000318147.

Protein family/group databases

CAZyiGH47. Glycoside Hydrolase Family 47.

PTM databases

iPTMnetiQ9BZQ6.
PhosphoSiteiQ9BZQ6.

Polymorphism and mutation databases

BioMutaiEDEM3.
DMDMi166897965.

Proteomic databases

EPDiQ9BZQ6.
MaxQBiQ9BZQ6.
PaxDbiQ9BZQ6.
PeptideAtlasiQ9BZQ6.
PRIDEiQ9BZQ6.

Protocols and materials databases

DNASUi80267.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi80267.
GeneCardsiEDEM3.
H-InvDBHIX0001417.
HGNCiHGNC:16787. EDEM3.
HPAiHPA025754.
HPA025755.
MIMi610214. gene.
neXtProtiNX_Q9BZQ6.
PharmGKBiPA38186.
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

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

Miscellaneous databases

ChiTaRSiEDEM3. human.
GeneWikiiEDEM3.
GenomeRNAii80267.
PROiQ9BZQ6.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000116406.
CleanExiHS_EDEM3.
ExpressionAtlasiQ9BZQ6. baseline and differential.
GenevisibleiQ9BZQ6. HS.

Family and domain databases

Gene3Di1.50.10.50. 1 hit.
InterProiIPR001382. Glyco_hydro_47.
IPR003137. PA_domain.
[Graphical view]
PfamiPF01532. Glyco_hydro_47. 1 hit.
PF02225. PA. 1 hit.
[Graphical view]
PRINTSiPR00747. GLYHDRLASE47.
SUPFAMiSSF48225. SSF48225. 1 hit.
PROSITEiPS00014. ER_TARGET. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiEDEM3_HUMAN
AccessioniPrimary (citable) accession number: Q9BZQ6
Secondary accession number(s): B2RCH6
, B7ZLZ2, Q0VGM5, Q5TEZ0, Q7L2Y5, Q9HCW1, Q9UFV7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 16, 2001
Last sequence update: February 5, 2008
Last modified: September 7, 2016
This is version 131 of the entry and version 2 of the sequence. [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. Glycosyl hydrolases
    Classification of glycosyl hydrolase families and list of entries
  2. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  7. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.