UniProtKB - Q13724 (MOGS_HUMAN)
Protein
Mannosyl-oligosaccharide glucosidase
Gene
MOGS
Organism
Homo sapiens (Human)
Status
Functioni
Cleaves the distal alpha 1,2-linked glucose residue from the Glc3Man9GlcNAc2 oligosaccharide precursor in a highly specific manner.
Catalytic activityi
- H2O + N4-(α-D-Glc-(1→2)-α-D-Glc-(1→3)-α-D-Glc-(1→3)-α-D-Man-(1→2)-α-D-Man-(1→2)-α-D-Man-(1→3)-[α-D-Man-(1→2)-α-D-Man-(1→3)-[α-D-Man-(1→2)-α-D-Man-(1→6)]-α-D-Man-(1→6)]-β-D-Man-(1→4)-β-D-GlcNAc-(1→4)-α-D-GlcNAc)-L-asparaginyl-[protein] = β-D-glucose + N4-(α-D-Glc-(1→3)-α-D-Glc-(1→3)-α-D-Man-(1→2)-α-D-Man-(1→2)-α-D-Man-(1→3)-[α-D-Man-(1→2)-α-D-Man-(1→3)-[α-D-Man-(1→2)-α-D-Man-(1→6)]-α-D-Man-(1→6)]-β-D-Man-(1→4)-β-D-GlcNAc-(1→4)-α-D-GlcNAc)-L-asparaginyl-[protein]EC:3.2.1.106
: N-glycan degradation Pathwayi
This protein is involved in the pathway N-glycan degradation, which is part of Glycan metabolism.View all proteins of this organism that are known to be involved in the pathway N-glycan degradation and in Glycan metabolism.
GO - Molecular functioni
- glucosidase activity Source: ProtInc
- mannosyl-oligosaccharide glucosidase activity Source: GO_Central
GO - Biological processi
- oligosaccharide metabolic process Source: InterPro
- protein folding Source: Reactome
- protein N-linked glycosylation Source: GO_Central
Keywordsi
Molecular function | Glycosidase, Hydrolase |
Enzyme and pathway databases
BioCyci | MetaCyc:HS03863-MONOMER |
PathwayCommonsi | Q13724 |
Reactomei | R-HSA-4793954, Defective MOGS causes MOGS-CDG (CDG-2b) R-HSA-532668, N-glycan trimming in the ER and Calnexin/Calreticulin cycle R-HSA-9683686, Maturation of spike protein R-HSA-9694548, Maturation of spike protein |
UniPathwayi | UPA00280 |
Protein family/group databases
CAZyi | GH63, Glycoside Hydrolase Family 63 |
Names & Taxonomyi
Protein namesi | Recommended name: Mannosyl-oligosaccharide glucosidase (EC:3.2.1.106)Alternative name(s): Processing A-glucosidase I |
Gene namesi | Name:MOGS Synonyms:GCS1 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:24862, MOGS |
MIMi | 601336, gene |
neXtProti | NX_Q13724 |
VEuPathDBi | HostDB:ENSG00000115275.11 |
Subcellular locationi
Endoplasmic reticulum
Endoplasmic reticulum
- endoplasmic reticulum Source: HPA
- endoplasmic reticulum membrane Source: GO_Central
Extracellular region or secreted
- extracellular exosome Source: UniProtKB
Other locations
- integral component of membrane Source: UniProtKB-KW
- membrane Source: UniProtKB
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 1 – 38 | CytoplasmicSequence analysisAdd BLAST | 38 | |
Transmembranei | 39 – 59 | Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST | 21 | |
Topological domaini | 60 – 837 | LumenalSequence analysisAdd BLAST | 778 |
Keywords - Cellular componenti
Endoplasmic reticulum, MembranePathology & Biotechi
Involvement in diseasei
Type IIb congenital disorder of glycosylation (CDGIIb)1 Publication
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionCharacterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms of the infant included hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course was progressive and the infant did not survive more than a few months.
Related information in OMIMFeature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_018966 | 486 | R → T in CDGIIb; loss of activity. 2 PublicationsCorresponds to variant dbSNP:rs121909291EnsemblClinVar. | 1 | |
Natural variantiVAR_018967 | 652 | F → L in CDGIIb; loss of activity. 2 PublicationsCorresponds to variant dbSNP:rs121909292EnsemblClinVar. | 1 |
Keywords - Diseasei
Disease variantOrganism-specific databases
DisGeNETi | 7841 |
GeneReviewsi | MOGS |
MalaCardsi | MOGS |
MIMi | 606056, phenotype |
OpenTargetsi | ENSG00000115275 |
Orphaneti | 79330, MOGS-CDG |
PharmGKBi | PA164723075 |
Miscellaneous databases
Pharosi | Q13724, Tbio |
Chemistry databases
ChEMBLi | CHEMBL4684 |
Genetic variation databases
BioMutai | MOGS |
DMDMi | 116242490 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000057710 | 1 – 837 | Mannosyl-oligosaccharide glucosidaseAdd BLAST | 837 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Glycosylationi | 657 | N-linked (GlcNAc...) asparagine | 1 |
Keywords - PTMi
GlycoproteinProteomic databases
EPDi | Q13724 |
jPOSTi | Q13724 |
MassIVEi | Q13724 |
MaxQBi | Q13724 |
PaxDbi | Q13724 |
PeptideAtlasi | Q13724 |
PRIDEi | Q13724 |
ProteomicsDBi | 27150 59667 [Q13724-1] |
PTM databases
GlyConnecti | 1489, 6 N-Linked glycans (1 site) |
GlyGeni | Q13724, 1 site, 1 N-linked glycan (1 site) |
iPTMneti | Q13724 |
PhosphoSitePlusi | Q13724 |
SwissPalmi | Q13724 |
Expressioni
Gene expression databases
Bgeei | ENSG00000115275, Expressed in left lobe of thyroid gland and 175 other tissues |
ExpressionAtlasi | Q13724, baseline and differential |
Genevisiblei | Q13724, HS |
Organism-specific databases
HPAi | ENSG00000115275, Low tissue specificity |
Interactioni
Protein-protein interaction databases
BioGRIDi | 113599, 116 interactors |
ELMi | Q13724 |
IntActi | Q13724, 33 interactors |
MINTi | Q13724 |
STRINGi | 9606.ENSP00000233616 |
Miscellaneous databases
RNActi | Q13724, protein |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 76 – 137 | Required for endoplasmic reticulum targetingBy similarityAdd BLAST | 62 |
Motif
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Motifi | 3 – 9 | Endoplasmic reticulum targeting | 7 |
Sequence similaritiesi
Belongs to the glycosyl hydrolase 63 family.Curated
Keywords - Domaini
Signal-anchor, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG2161, Eukaryota |
GeneTreei | ENSGT00390000017452 |
HOGENOMi | CLU_007380_1_0_1 |
InParanoidi | Q13724 |
OMAi | PLYMKRN |
OrthoDBi | 278028at2759 |
PhylomeDBi | Q13724 |
TreeFami | TF300749 |
Family and domain databases
Gene3Di | 1.50.10.10, 1 hit 2.70.98.110, 1 hit |
InterProi | View protein in InterPro IPR008928, 6-hairpin_glycosidase_sf IPR012341, 6hp_glycosidase-like_sf IPR031335, Glyco_hydro_63_C IPR031631, Glyco_hydro_63N IPR038518, Glyco_hydro_63N_sf IPR004888, Glycoside_hydrolase_63 |
PANTHERi | PTHR10412, PTHR10412, 1 hit |
Pfami | View protein in Pfam PF03200, Glyco_hydro_63, 1 hit PF16923, Glyco_hydro_63N, 1 hit |
SUPFAMi | SSF48208, SSF48208, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 11 potential isoforms that are computationally mapped.Show allAlign All
Isoform 1 (identifier: Q13724-1) [UniParc]FASTAAdd to basket
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MARGERRRRA VPAEGVRTAE RAARGGPGRR DGRGGGPRST AGGVALAVVV
60 70 80 90 100
LSLALGMSGR WVLAWYRARR AVTLHSAPPV LPADSSSPAV APDLFWGTYR
110 120 130 140 150
PHVYFGMKTR SPKPLLTGLM WAQQGTTPGT PKLRHTCEQG DGVGPYGWEF
160 170 180 190 200
HDGLSFGRQH IQDGALRLTT EFVKRPGGQH GGDWSWRVTV EPQDSGTSAL
210 220 230 240 250
PLVSLFFYVV TDGKEVLLPE VGAKGQLKFI SGHTSELGDF RFTLLPPTSP
260 270 280 290 300
GDTAPKYGSY NVFWTSNPGL PLLTEMVKSR LNSWFQHRPP GAPPERYLGL
310 320 330 340 350
PGSLKWEDRG PSGQGQGQFL IQQVTLKIPI SIEFVFESGS AQAGGNQALP
360 370 380 390 400
RLAGSLLTQA LESHAEGFRE RFEKTFQLKE KGLSSGEQVL GQAALSGLLG
410 420 430 440 450
GIGYFYGQGL VLPDIGVEGS EQKVDPALFP PVPLFTAVPS RSFFPRGFLW
460 470 480 490 500
DEGFHQLVVQ RWDPSLTREA LGHWLGLLNA DGWIGREQIL GDEARARVPP
510 520 530 540 550
EFLVQRAVHA NPPTLLLPVA HMLEVGDPDD LAFLRKALPR LHAWFSWLHQ
560 570 580 590 600
SQAGPLPLSY RWRGRDPALP TLLNPKTLPS GLDDYPRASH PSVTERHLDL
610 620 630 640 650
RCWVALGARV LTRLAEHLGE AEVAAELGPL AASLEAAESL DELHWAPELG
660 670 680 690 700
VFADFGNHTK AVQLKPRPPQ GLVRVVGRPQ PQLQYVDALG YVSLFPLLLR
710 720 730 740 750
LLDPTSSRLG PLLDILADSR HLWSPFGLRS LAASSSFYGQ RNSEHDPPYW
760 770 780 790 800
RGAVWLNVNY LALGALHHYG HLEGPHQARA AKLHGELRAN VVGNVWRQYQ
810 820 830
ATGFLWEQYS DRDGRGMGCR PFHGWTSLVL LAMAEDY
Computationally mapped potential isoform sequencesi
There are 11 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketA0A3B3IS52 | A0A3B3IS52_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 428 | Annotation score: | ||
B8ZZE2 | B8ZZE2_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 209 | Annotation score: | ||
A0A3B3ITC1 | A0A3B3ITC1_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 294 | Annotation score: | ||
A0A3B3ITU6 | A0A3B3ITU6_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 208 | Annotation score: | ||
A0A3B3IRK6 | A0A3B3IRK6_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 715 | Annotation score: | ||
B4E3B8 | B4E3B8_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 159 | Annotation score: | ||
A0A3B3IRW2 | A0A3B3IRW2_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 562 | Annotation score: | ||
A0A3B3IRI7 | A0A3B3IRI7_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 113 | Annotation score: | ||
C9JDQ1 | C9JDQ1_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 145 | Annotation score: | ||
A0A3B3IRI1 | A0A3B3IRI1_HUMAN | Mannosyl-oligosaccharide glucosidas... | MOGS | 75 | Annotation score: | ||
There is more potential isoformShow all |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 330 | I → F in CAA60683 (PubMed:7635146).Curated | 1 | |
Sequence conflicti | 389 | V → A in CAA60683 (PubMed:7635146).Curated | 1 | |
Sequence conflicti | 605 | A → G in CAA60683 (PubMed:7635146).Curated | 1 | |
Sequence conflicti | 818 | Missing in CAA60683 (PubMed:7635146).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_049233 | 222 | G → R. Corresponds to variant dbSNP:rs3213671EnsemblClinVar. | 1 | |
Natural variantiVAR_019361 | 236 | E → Q1 PublicationCorresponds to variant dbSNP:rs1063587Ensembl. | 1 | |
Natural variantiVAR_019362 | 239 | D → N1 PublicationCorresponds to variant dbSNP:rs1063588EnsemblClinVar. | 1 | |
Natural variantiVAR_049234 | 293 | P → S1 PublicationCorresponds to variant dbSNP:rs2268416EnsemblClinVar. | 1 | |
Natural variantiVAR_018966 | 486 | R → T in CDGIIb; loss of activity. 2 PublicationsCorresponds to variant dbSNP:rs121909291EnsemblClinVar. | 1 | |
Natural variantiVAR_049235 | 495 | R → P. Corresponds to variant dbSNP:rs34075781Ensembl. | 1 | |
Natural variantiVAR_018967 | 652 | F → L in CDGIIb; loss of activity. 2 PublicationsCorresponds to variant dbSNP:rs121909292EnsemblClinVar. | 1 | |
Natural variantiVAR_049236 | 785 | G → S. Corresponds to variant dbSNP:rs35533773EnsemblClinVar. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_046921 | 1 – 106 | Missing in isoform 2. CuratedAdd BLAST | 106 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X87237 mRNA Translation: CAA60683.1 AJ422288 Genomic DNA Translation: CAD19636.1 AK292553 mRNA Translation: BAF85242.1 AC005041 Genomic DNA No translation available. CH471053 Genomic DNA Translation: EAW99653.1 BC117252 mRNA Translation: AAI17253.1 BC117250 mRNA Translation: AAI17251.1 |
CCDSi | CCDS42700.1 [Q13724-1] CCDS54370.1 [Q13724-2] |
PIRi | S66258 |
RefSeqi | NP_001139630.1, NM_001146158.1 [Q13724-2] NP_006293.2, NM_006302.2 [Q13724-1] |
Genome annotation databases
Ensembli | ENST00000448666; ENSP00000410992; ENSG00000115275 [Q13724-1] ENST00000452063; ENSP00000388201; ENSG00000115275 [Q13724-2] |
GeneIDi | 7841 |
KEGGi | hsa:7841 |
UCSCi | uc010ffi.4, human [Q13724-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | X87237 mRNA Translation: CAA60683.1 AJ422288 Genomic DNA Translation: CAD19636.1 AK292553 mRNA Translation: BAF85242.1 AC005041 Genomic DNA No translation available. CH471053 Genomic DNA Translation: EAW99653.1 BC117252 mRNA Translation: AAI17253.1 BC117250 mRNA Translation: AAI17251.1 |
CCDSi | CCDS42700.1 [Q13724-1] CCDS54370.1 [Q13724-2] |
PIRi | S66258 |
RefSeqi | NP_001139630.1, NM_001146158.1 [Q13724-2] NP_006293.2, NM_006302.2 [Q13724-1] |
3D structure databases
SMRi | Q13724 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 113599, 116 interactors |
ELMi | Q13724 |
IntActi | Q13724, 33 interactors |
MINTi | Q13724 |
STRINGi | 9606.ENSP00000233616 |
Chemistry databases
ChEMBLi | CHEMBL4684 |
Protein family/group databases
CAZyi | GH63, Glycoside Hydrolase Family 63 |
PTM databases
GlyConnecti | 1489, 6 N-Linked glycans (1 site) |
GlyGeni | Q13724, 1 site, 1 N-linked glycan (1 site) |
iPTMneti | Q13724 |
PhosphoSitePlusi | Q13724 |
SwissPalmi | Q13724 |
Genetic variation databases
BioMutai | MOGS |
DMDMi | 116242490 |
Proteomic databases
EPDi | Q13724 |
jPOSTi | Q13724 |
MassIVEi | Q13724 |
MaxQBi | Q13724 |
PaxDbi | Q13724 |
PeptideAtlasi | Q13724 |
PRIDEi | Q13724 |
ProteomicsDBi | 27150 59667 [Q13724-1] |
Protocols and materials databases
Antibodypediai | 2372, 180 antibodies |
Genome annotation databases
Ensembli | ENST00000448666; ENSP00000410992; ENSG00000115275 [Q13724-1] ENST00000452063; ENSP00000388201; ENSG00000115275 [Q13724-2] |
GeneIDi | 7841 |
KEGGi | hsa:7841 |
UCSCi | uc010ffi.4, human [Q13724-1] |
Organism-specific databases
CTDi | 7841 |
DisGeNETi | 7841 |
GeneCardsi | MOGS |
GeneReviewsi | MOGS |
HGNCi | HGNC:24862, MOGS |
HPAi | ENSG00000115275, Low tissue specificity |
MalaCardsi | MOGS |
MIMi | 601336, gene 606056, phenotype |
neXtProti | NX_Q13724 |
OpenTargetsi | ENSG00000115275 |
Orphaneti | 79330, MOGS-CDG |
PharmGKBi | PA164723075 |
VEuPathDBi | HostDB:ENSG00000115275.11 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG2161, Eukaryota |
GeneTreei | ENSGT00390000017452 |
HOGENOMi | CLU_007380_1_0_1 |
InParanoidi | Q13724 |
OMAi | PLYMKRN |
OrthoDBi | 278028at2759 |
PhylomeDBi | Q13724 |
TreeFami | TF300749 |
Enzyme and pathway databases
UniPathwayi | UPA00280 |
BioCyci | MetaCyc:HS03863-MONOMER |
PathwayCommonsi | Q13724 |
Reactomei | R-HSA-4793954, Defective MOGS causes MOGS-CDG (CDG-2b) R-HSA-532668, N-glycan trimming in the ER and Calnexin/Calreticulin cycle R-HSA-9683686, Maturation of spike protein R-HSA-9694548, Maturation of spike protein |
Miscellaneous databases
BioGRID-ORCSi | 7841, 48 hits in 875 CRISPR screens |
ChiTaRSi | MOGS, human |
GeneWikii | GCS1 |
GenomeRNAii | 7841 |
Pharosi | Q13724, Tbio |
PROi | PR:Q13724 |
RNActi | Q13724, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000115275, Expressed in left lobe of thyroid gland and 175 other tissues |
ExpressionAtlasi | Q13724, baseline and differential |
Genevisiblei | Q13724, HS |
Family and domain databases
Gene3Di | 1.50.10.10, 1 hit 2.70.98.110, 1 hit |
InterProi | View protein in InterPro IPR008928, 6-hairpin_glycosidase_sf IPR012341, 6hp_glycosidase-like_sf IPR031335, Glyco_hydro_63_C IPR031631, Glyco_hydro_63N IPR038518, Glyco_hydro_63N_sf IPR004888, Glycoside_hydrolase_63 |
PANTHERi | PTHR10412, PTHR10412, 1 hit |
Pfami | View protein in Pfam PF03200, Glyco_hydro_63, 1 hit PF16923, Glyco_hydro_63N, 1 hit |
SUPFAMi | SSF48208, SSF48208, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | MOGS_HUMAN | |
Accessioni | Q13724Primary (citable) accession number: Q13724 Secondary accession number(s): A8K938 Q8TCT5 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | November 1, 1997 |
Last sequence update: | October 17, 2006 | |
Last modified: | February 10, 2021 | |
This is version 191 of the entry and version 5 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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
Reference proteomeDocuments
- Glycosyl hydrolases
Classification of glycosyl hydrolase families and list of entries - Human chromosome 2
Human chromosome 2: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - SIMILARITY comments
Index of protein domains and families