Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Protein ERGIC-53

Gene

LMAN1

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

Functioni

Mannose-specific lectin. May recognize sugar residues of glycoproteins, glycolipids, or glycosylphosphatidyl inositol anchors and may be involved in the sorting or recycling of proteins, lipids, or both. The LMAN1-MCFD2 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins.2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei88CarbohydratePROSITE-ProRule annotation1
Binding sitei121CarbohydratePROSITE-ProRule annotation1
Metal bindingi152Calcium1
Metal bindingi154Calcium; via carbonyl oxygen1
Metal bindingi156Calcium1
Binding sitei156CarbohydratePROSITE-ProRule annotation1
Binding sitei178CarbohydratePROSITE-ProRule annotation1
Metal bindingi181Calcium1
Sitei501Required for ER export1

GO - Molecular functioni

  • mannose binding Source: ProtInc
  • metal ion binding Source: UniProtKB-KW
  • unfolded protein binding Source: ProtInc

GO - Biological processi

  • blood coagulation Source: ProtInc
  • COPII vesicle coating Source: Reactome
  • endoplasmic reticulum organization Source: GO_Central
  • ER to Golgi vesicle-mediated transport Source: Reactome
  • Golgi organization Source: UniProtKB
  • positive regulation of organelle organization Source: UniProtKB
  • protein folding Source: ProtInc
  • protein N-linked glycosylation via asparagine Source: Reactome
  • protein transport Source: UniProtKB-KW
Complete GO annotation...

Keywords - Biological processi

ER-Golgi transport, Protein transport, Transport

Keywords - Ligandi

Lectin, Metal-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000074695-MONOMER.
ReactomeiR-HSA-204005. COPII (Coat Protein 2) Mediated Vesicle Transport.
R-HSA-5694530. Cargo concentration in the ER.
R-HSA-948021. Transport to the Golgi and subsequent modification.

Names & Taxonomyi

Protein namesi
Recommended name:
Protein ERGIC-53
Alternative name(s):
ER-Golgi intermediate compartment 53 kDa protein
Gp58
Intracellular mannose-specific lectin MR60
Lectin mannose-binding 1
Gene namesi
Name:LMAN1
Synonyms:ERGIC53, F5F8D
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 18

Organism-specific databases

HGNCiHGNC:6631. LMAN1.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini31 – 477LumenalSequence analysisAdd BLAST447
Transmembranei478 – 498HelicalSequence analysisAdd BLAST21
Topological domaini499 – 510CytoplasmicSequence analysisAdd BLAST12

GO - Cellular componenti

  • endoplasmic reticulum-Golgi intermediate compartment Source: UniProtKB
  • endoplasmic reticulum-Golgi intermediate compartment membrane Source: Reactome
  • endoplasmic reticulum membrane Source: Reactome
  • ER to Golgi transport vesicle Source: GO_Central
  • ER to Golgi transport vesicle membrane Source: Reactome
  • extracellular exosome Source: UniProtKB
  • Golgi membrane Source: ProtInc
  • host cell perinuclear region of cytoplasm Source: UniProtKB
  • integral component of membrane Source: ProtInc
  • membrane Source: UniProtKB
  • sarcomere Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum, Golgi apparatus, Membrane

Pathology & Biotechi

Involvement in diseasei

Factor V and factor VIII combined deficiency 1 (F5F8D1)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA blood coagulation disorder characterized by bleeding symptoms similar to those in hemophilia or parahemophilia, that are caused by single deficiency of FV or FVIII, respectively. The most common symptoms are epistaxis, menorrhagia, and excessive bleeding during or after trauma. Plasma levels of coagulation factors V and VIII are in the range of 5 to 30% of normal.
See also OMIM:227300
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07196967W → S in F5F8D1; loss of interaction with MCFD2 and ability to bind D-mannose. 1 Publication1

Organism-specific databases

DisGeNETi3998.
MalaCardsiLMAN1.
MIMi227300. phenotype.
OpenTargetsiENSG00000074695.
Orphaneti35909. Combined deficiency of factor V and factor VIII.
PharmGKBiPA30399.

Chemistry databases

DrugBankiDB00025. Antihemophilic Factor (Recombinant).

Polymorphism and mutation databases

BioMutaiLMAN1.
DMDMi22261801.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 30Combined sources3 PublicationsAdd BLAST30
ChainiPRO_000001766031 – 510Protein ERGIC-53Add BLAST480

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi190 ↔ 230
Modified residuei425PhosphoserineCombined sources1
Disulfide bondi466Interchain
Disulfide bondi475Interchain

Post-translational modificationi

The N-terminal may be partly blocked.

Keywords - PTMi

Disulfide bond, Phosphoprotein

Proteomic databases

EPDiP49257.
MaxQBiP49257.
PaxDbiP49257.
PeptideAtlasiP49257.
PRIDEiP49257.

PTM databases

iPTMnetiP49257.
PhosphoSitePlusiP49257.
SwissPalmiP49257.

Expressioni

Tissue specificityi

Ubiquitous.1 Publication

Gene expression databases

BgeeiENSG00000074695.
ExpressionAtlasiP49257. baseline and differential.
GenevisibleiP49257. HS.

Organism-specific databases

HPAiCAB037163.
HPA002320.

Interactioni

Subunit structurei

Exists both as a covalent disulfide-linked homohexamer, and a complex of three disulfide-linked dimers non-covalently kept together. Interacts with MCFD2. May interact with TMEM115.7 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
ERP44Q9BS263EBI-1057738,EBI-541644
SURF4O152603EBI-1057738,EBI-1044848

GO - Molecular functioni

  • unfolded protein binding Source: ProtInc

Protein-protein interaction databases

BioGridi110185. 67 interactors.
DIPiDIP-42188N.
IntActiP49257. 14 interactors.
MINTiMINT-4999949.
STRINGi9606.ENSP00000251047.

Structurei

Secondary structure

1510
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi43 – 46Combined sources4
Helixi48 – 50Combined sources3
Beta strandi52 – 56Combined sources5
Beta strandi67 – 71Combined sources5
Beta strandi80 – 83Combined sources4
Beta strandi85 – 88Combined sources4
Beta strandi90 – 97Combined sources8
Beta strandi102 – 113Combined sources12
Beta strandi115 – 118Combined sources4
Beta strandi122 – 128Combined sources7
Beta strandi134 – 137Combined sources4
Beta strandi146 – 152Combined sources7
Beta strandi158 – 160Combined sources3
Beta strandi164 – 170Combined sources7
Helixi178 – 180Combined sources3
Helixi183 – 185Combined sources3
Beta strandi186 – 191Combined sources6
Beta strandi201 – 208Combined sources8
Beta strandi211 – 217Combined sources7
Beta strandi219 – 222Combined sources4
Beta strandi228 – 235Combined sources8
Beta strandi240 – 249Combined sources10
Beta strandi251 – 253Combined sources3
Beta strandi256 – 268Combined sources13

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3A4UX-ray1.84A31-285[»]
3LCPX-ray2.45A/B32-277[»]
3WHTX-ray1.80A31-269[»]
3WHUX-ray2.60A31-269[»]
3WNXX-ray2.75A31-269[»]
4GKXX-ray2.70A/B/C/D/E/F31-270[»]
4GKYX-ray2.42A31-270[»]
4YGBX-ray1.60A/C31-269[»]
4YGCX-ray2.40A/C/E/G31-269[»]
4YGDX-ray2.51A/C/E/G31-269[»]
4YGEX-ray3.05A/C/E31-269[»]
ProteinModelPortaliP49257.
SMRiP49257.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP49257.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini44 – 267L-type lectin-likePROSITE-ProRule annotationAdd BLAST224

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni251 – 253Carbohydrate bindingPROSITE-ProRule annotation3

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi509 – 510ER export motif2

Domaini

The FF ER export motif at the C-terminus is not sufficient to support endoplasmic reticulum exit, and needs assistance of Gln-501 for proper recognition of COPII coat components.

Sequence similaritiesi

Contains 1 L-type lectin-like domain.PROSITE-ProRule annotation

Keywords - Domaini

Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3838. Eukaryota.
ENOG410ZFRX. LUCA.
GeneTreeiENSGT00530000062977.
HOVERGENiHBG052332.
InParanoidiP49257.
KOiK10080.
OMAiNMIIPAQ.
OrthoDBiEOG091G08WZ.
PhylomeDBiP49257.
TreeFamiTF313311.

Family and domain databases

Gene3Di2.60.120.200. 1 hit.
InterProiIPR013320. ConA-like_dom.
IPR005052. Lectin_leg.
IPR033085. LMAN1.
[Graphical view]
PANTHERiPTHR12223:SF32. PTHR12223:SF32. 1 hit.
PfamiPF03388. Lectin_leg-like. 1 hit.
[Graphical view]
SUPFAMiSSF49899. SSF49899. 1 hit.
PROSITEiPS51328. L_LECTIN_LIKE. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

P49257-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAGSRQRGLR ARVRPLFCAL LLSLGRFVRG DGVGGDPAVA LPHRRFEYKY
60 70 80 90 100
SFKGPHLVQS DGTVPFWAHA GNAIPSSDQI RVAPSLKSQR GSVWTKTKAA
110 120 130 140 150
FENWEVEVTF RVTGRGRIGA DGLAIWYAEN QGLEGPVFGS ADLWNGVGIF
160 170 180 190 200
FDSFDNDGKK NNPAIVIIGN NGQIHYDHQN DGASQALASC QRDFRNKPYP
210 220 230 240 250
VRAKITYYQN TLTVMINNGF TPDKNDYEFC AKVENMIIPA QGHFGISAAT
260 270 280 290 300
GGLADDHDVL SFLTFQLTEP GKEPPTPDKE ISEKEKEKYQ EEFEHFQQEL
310 320 330 340 350
DKKKEEFQKG HPDLQGQPAE EIFESVGDRE LRQVFEGQNR IHLEIKQLNR
360 370 380 390 400
QLDMILDEQR RYVSSLTEEI SKRGAGMPGQ HGQITQQELD TVVKTQHEIL
410 420 430 440 450
RQVNEMKNSM SETVRLVSGM QHPGSAGGVY ETTQHFIDIK EHLHIVKRDI
460 470 480 490 500
DNLVQRNMPS NEKPKCPELP PFPSCLSTVH FIIFVVVQTV LFIGYIMYRS
510
QQEAAAKKFF
Length:510
Mass (Da):57,549
Last modified:August 13, 2002 - v2
Checksum:iB87EF117C0CD386C
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti153S → T in CAA50653 (PubMed:8223692).Curated1

Mass spectrometryi

Molecular mass is 54222.91 Da from positions 31 - 510. Determined by MALDI. 1 Publication

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_01370314R → Q.1 PublicationCorresponds to variant rs1043302dbSNPEnsembl.1
Natural variantiVAR_01370439V → A.1 PublicationCorresponds to variant rs33926449dbSNPEnsembl.1
Natural variantiVAR_07196967W → S in F5F8D1; loss of interaction with MCFD2 and ability to bind D-mannose. 1 Publication1
Natural variantiVAR_049770355I → T.Corresponds to variant rs3737392dbSNPEnsembl.1
Natural variantiVAR_013705410M → L.2 PublicationsCorresponds to variant rs2298711dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X71661 mRNA. Translation: CAA50653.1.
U09716 mRNA. Translation: AAA95960.1.
AF081866, AF081865 Genomic DNA. Translation: AAD32479.1.
AF081867 Genomic DNA. Translation: AAD32480.1.
AF081869, AF081868 Genomic DNA. Translation: AAD32481.1.
AF081871, AF081870 Genomic DNA. Translation: AAD32482.1.
AF081873, AF081872 Genomic DNA. Translation: AAD32483.1.
AF081875, AF081874 Genomic DNA. Translation: AAD32484.1.
AF081877, AF081876 Genomic DNA. Translation: AAD32485.1.
AF081879, AF081878 Genomic DNA. Translation: AAD32486.1.
AF081880 Genomic DNA. Translation: AAD32487.1.
AF081882, AF081881 Genomic DNA. Translation: AAD32488.1.
AF081884, AF081883 Genomic DNA. Translation: AAD32489.1.
AF081885 Genomic DNA. Translation: AAD32490.1.
BC032330 mRNA. Translation: AAH32330.1.
CCDSiCCDS11974.1.
PIRiS42626.
RefSeqiNP_005561.1. NM_005570.3.
UniGeneiHs.465295.

Genome annotation databases

EnsembliENST00000251047; ENSP00000251047; ENSG00000074695.
GeneIDi3998.
KEGGihsa:3998.
UCSCiuc002lhz.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X71661 mRNA. Translation: CAA50653.1.
U09716 mRNA. Translation: AAA95960.1.
AF081866, AF081865 Genomic DNA. Translation: AAD32479.1.
AF081867 Genomic DNA. Translation: AAD32480.1.
AF081869, AF081868 Genomic DNA. Translation: AAD32481.1.
AF081871, AF081870 Genomic DNA. Translation: AAD32482.1.
AF081873, AF081872 Genomic DNA. Translation: AAD32483.1.
AF081875, AF081874 Genomic DNA. Translation: AAD32484.1.
AF081877, AF081876 Genomic DNA. Translation: AAD32485.1.
AF081879, AF081878 Genomic DNA. Translation: AAD32486.1.
AF081880 Genomic DNA. Translation: AAD32487.1.
AF081882, AF081881 Genomic DNA. Translation: AAD32488.1.
AF081884, AF081883 Genomic DNA. Translation: AAD32489.1.
AF081885 Genomic DNA. Translation: AAD32490.1.
BC032330 mRNA. Translation: AAH32330.1.
CCDSiCCDS11974.1.
PIRiS42626.
RefSeqiNP_005561.1. NM_005570.3.
UniGeneiHs.465295.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3A4UX-ray1.84A31-285[»]
3LCPX-ray2.45A/B32-277[»]
3WHTX-ray1.80A31-269[»]
3WHUX-ray2.60A31-269[»]
3WNXX-ray2.75A31-269[»]
4GKXX-ray2.70A/B/C/D/E/F31-270[»]
4GKYX-ray2.42A31-270[»]
4YGBX-ray1.60A/C31-269[»]
4YGCX-ray2.40A/C/E/G31-269[»]
4YGDX-ray2.51A/C/E/G31-269[»]
4YGEX-ray3.05A/C/E31-269[»]
ProteinModelPortaliP49257.
SMRiP49257.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110185. 67 interactors.
DIPiDIP-42188N.
IntActiP49257. 14 interactors.
MINTiMINT-4999949.
STRINGi9606.ENSP00000251047.

Chemistry databases

DrugBankiDB00025. Antihemophilic Factor (Recombinant).

PTM databases

iPTMnetiP49257.
PhosphoSitePlusiP49257.
SwissPalmiP49257.

Polymorphism and mutation databases

BioMutaiLMAN1.
DMDMi22261801.

Proteomic databases

EPDiP49257.
MaxQBiP49257.
PaxDbiP49257.
PeptideAtlasiP49257.
PRIDEiP49257.

Protocols and materials databases

DNASUi3998.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000251047; ENSP00000251047; ENSG00000074695.
GeneIDi3998.
KEGGihsa:3998.
UCSCiuc002lhz.4. human.

Organism-specific databases

CTDi3998.
DisGeNETi3998.
GeneCardsiLMAN1.
HGNCiHGNC:6631. LMAN1.
HPAiCAB037163.
HPA002320.
MalaCardsiLMAN1.
MIMi227300. phenotype.
601567. gene.
neXtProtiNX_P49257.
OpenTargetsiENSG00000074695.
Orphaneti35909. Combined deficiency of factor V and factor VIII.
PharmGKBiPA30399.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3838. Eukaryota.
ENOG410ZFRX. LUCA.
GeneTreeiENSGT00530000062977.
HOVERGENiHBG052332.
InParanoidiP49257.
KOiK10080.
OMAiNMIIPAQ.
OrthoDBiEOG091G08WZ.
PhylomeDBiP49257.
TreeFamiTF313311.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000074695-MONOMER.
ReactomeiR-HSA-204005. COPII (Coat Protein 2) Mediated Vesicle Transport.
R-HSA-5694530. Cargo concentration in the ER.
R-HSA-948021. Transport to the Golgi and subsequent modification.

Miscellaneous databases

ChiTaRSiLMAN1. human.
EvolutionaryTraceiP49257.
GeneWikiiLMAN1.
GenomeRNAii3998.
PROiP49257.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000074695.
ExpressionAtlasiP49257. baseline and differential.
GenevisibleiP49257. HS.

Family and domain databases

Gene3Di2.60.120.200. 1 hit.
InterProiIPR013320. ConA-like_dom.
IPR005052. Lectin_leg.
IPR033085. LMAN1.
[Graphical view]
PANTHERiPTHR12223:SF32. PTHR12223:SF32. 1 hit.
PfamiPF03388. Lectin_leg-like. 1 hit.
[Graphical view]
SUPFAMiSSF49899. SSF49899. 1 hit.
PROSITEiPS51328. L_LECTIN_LIKE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiLMAN1_HUMAN
AccessioniPrimary (citable) accession number: P49257
Secondary accession number(s): Q12895
, Q8N5I7, Q9UQG1, Q9UQG2, Q9UQG3, Q9UQG4, Q9UQG5, Q9UQG6, Q9UQG7, Q9UQG8, Q9UQG9, Q9UQH0, Q9UQH1, Q9UQH2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: August 13, 2002
Last modified: November 2, 2016
This is version 173 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

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 18
    Human chromosome 18: 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
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. 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.