GlyCosmos Portal

Annotation

Keyword

Alternative splicing
Calcium
Congenital disorder of glycosylation
Endoplasmic reticulum
Glycoprotein
Glycosidase
Hydrolase
Metal-binding
Proteomics identification
Reference proteome
Signal
Unfolded protein response

Proteins

Displaying **all 2** entries
UniProt	Protein Name
A0A8J8YX80
Q9BZQ6	Alpha-1,2-mannosidase EDEM3

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 10 in total

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GO Term	Evidence Code	PMID
carbohydrate metabolic process	IEA
mannoprotein catabolic process	IMP	25092655
mannoprotein catabolic process	IBA
protein glycosylation	IEA
response to unfolded protein

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

organonitrogen compound metabolic process [inference]

protein metabolic process [inference]

proteolysis [inference]

proteolysis involved in protein catabolic process [inference]

mannoprotein catabolic process

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

modification-dependent protein catabolic process [inference]

ubiquitin-dependent protein catabolic process [inference]

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

glycoprotein metabolic process [inference]

mannoprotein metabolic process [inference]

mannoprotein catabolic process

glycoprotein catabolic process [inference]

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

protein modification process [inference]

protein glycosylation

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

organonitrogen compound catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

catabolic process [inference]

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

modification-dependent macromolecule catabolic process [inference]

modification-dependent protein catabolic process [inference]

ubiquitin-dependent protein catabolic process [inference]

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

carbohydrate derivative catabolic process [inference]

glycoprotein catabolic process [inference]

organonitrogen compound catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

primary metabolic process [inference]

carbohydrate metabolic process

protein metabolic process [inference]

proteolysis [inference]

proteolysis involved in protein catabolic process [inference]

mannoprotein catabolic process

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

modification-dependent protein catabolic process [inference]

ubiquitin-dependent protein catabolic process [inference]

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

glycoprotein metabolic process [inference]

mannoprotein metabolic process [inference]

mannoprotein catabolic process

glycoprotein catabolic process [inference]

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

protein modification process [inference]

protein glycosylation

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

glycosylation [inference]

macromolecule glycosylation [inference]

protein glycosylation

organic substance metabolic process [inference]

carbohydrate metabolic process

macromolecule metabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

modification-dependent macromolecule catabolic process [inference]

modification-dependent protein catabolic process [inference]

ubiquitin-dependent protein catabolic process [inference]

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

protein metabolic process [inference]

proteolysis [inference]

proteolysis involved in protein catabolic process [inference]

mannoprotein catabolic process

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

modification-dependent protein catabolic process [inference]

ubiquitin-dependent protein catabolic process [inference]

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

glycoprotein metabolic process [inference]

mannoprotein metabolic process [inference]

mannoprotein catabolic process

glycoprotein catabolic process [inference]

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

protein modification process [inference]

protein glycosylation

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

macromolecule modification [inference]

protein modification process [inference]

protein glycosylation

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

macromolecule glycosylation [inference]

protein glycosylation

carbohydrate derivative metabolic process [inference]

glycoprotein metabolic process [inference]

mannoprotein metabolic process [inference]

mannoprotein catabolic process

glycoprotein catabolic process [inference]

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

carbohydrate derivative catabolic process [inference]

glycoprotein catabolic process [inference]

organonitrogen compound metabolic process [inference]

protein metabolic process [inference]

proteolysis [inference]

proteolysis involved in protein catabolic process [inference]

mannoprotein catabolic process

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

modification-dependent protein catabolic process [inference]

ubiquitin-dependent protein catabolic process [inference]

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

glycoprotein metabolic process [inference]

mannoprotein metabolic process [inference]

mannoprotein catabolic process

glycoprotein catabolic process [inference]

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

protein modification process [inference]

protein glycosylation

protein deglycosylation [inference]

protein deglycosylation involved in glycoprotein catabolic process [inference]

mannose trimming involved in glycoprotein ERAD pathway

protein demannosylation [inference]

protein alpha-1,2-demannosylation [inference]

organonitrogen compound catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

modification-dependent macromolecule catabolic process [inference]

modification-dependent protein catabolic process [inference]

ubiquitin-dependent protein catabolic process [inference]

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

carbohydrate derivative catabolic process [inference]

glycoprotein catabolic process [inference]

organonitrogen compound catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteasomal protein catabolic process [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

proteasome-mediated ubiquitin-dependent protein catabolic process [inference]

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

cellular process [inference]

signal transduction [inference]

endoplasmic reticulum unfolded protein response

cellular response to stimulus [inference]

cellular response to stress [inference]

response to endoplasmic reticulum stress [inference]

endoplasmic reticulum unfolded protein response

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

response to stimulus [inference]

response to stress [inference]

cellular response to stress [inference]

response to endoplasmic reticulum stress [inference]

endoplasmic reticulum unfolded protein response

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

response to topologically incorrect protein [inference]

response to unfolded protein

response to chemical [inference]

response to organic substance [inference]

response to organonitrogen compound [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

response to glycoprotein [inference]

ubiquitin-dependent glycoprotein ERAD pathway

response to topologically incorrect protein [inference]

response to unfolded protein

response to nitrogen compound [inference]

response to organonitrogen compound [inference]

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

response to glycoprotein [inference]

ubiquitin-dependent glycoprotein ERAD pathway

response to oxygen-containing compound [inference]

response to glycoprotein [inference]

ubiquitin-dependent glycoprotein ERAD pathway

cellular response to stimulus [inference]

cellular response to stress [inference]

response to endoplasmic reticulum stress [inference]

endoplasmic reticulum unfolded protein response

ERAD pathway

ubiquitin-dependent ERAD pathway [inference]

ubiquitin-dependent glycoprotein ERAD pathway

biological regulation [inference]

regulation of biological process [inference]

regulation of cellular process [inference]

signal transduction [inference]

endoplasmic reticulum unfolded protein response

Displaying **all 4** entries
GO Term	Evidence Code	PMID
endoplasmic reticulum	IBA
endoplasmic reticulum lumen	IEA
membrane	IEA
endoplasmic reticulum quality control compartment	TAS

GO Hierarchy

cellular component

cellular anatomical entity [inference]

membrane

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

endoplasmic reticulum lumen

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

endoplasmic reticulum

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

endoplasmic reticulum

endoplasmic reticulum quality control compartment

Displaying **all 4** entries
GO Term	Evidence Code	PMID
mannosyl-oligosaccharide 1,2-alpha-mannosidase activity	TAS
mannosyl-oligosaccharide 1,2-alpha-mannosidase activity	IMP	25092655
mannosyl-oligosaccharide 1,2-alpha-mannosidase activity	IBA
calcium ion binding	IEA

GO Hierarchy

molecular function

catalytic activity [inference]

hydrolase activity [inference]

hydrolase activity, acting on glycosyl bonds [inference]

hydrolase activity, hydrolyzing O-glycosyl compounds [inference]

mannosidase activity [inference]

alpha-mannosidase activity [inference]

mannosyl-oligosaccharide mannosidase activity [inference]

mannosyl-oligosaccharide 1,2-alpha-mannosidase activity

binding [inference]

ion binding [inference]

cation binding [inference]

metal ion binding [inference]

calcium ion binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

Mannosyl-oligosaccharide

Functional Category

E: Amino acid transport and metabolism
K: Transcription
T: Signal transduction mechanisms

Displaying **all 5** entries
Gene Ontology
calcium ion binding
hydrolase activity, acting on glycosyl bonds
hydrolase activity
mannosyl-oligosaccharide 1,2-alpha-mannosidase activity
metal ion binding

Displaying **all 3** entries
InterPro
Glycoside hydrolase family 47
Seven-hairpin glycosidases
Six-hairpin glycosidase-like superfamily

Human Protein Atlas

ENSG00000116406

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K10086

Name

ER degradation enhancer, mannosidase alpha-like 3

References

16431915

Rhea

RHEA:56028

H₂O

β-D-mannose

Enzyme

3.2.1.113

PMID

RHEA:56008

H₂O

β-D-mannose

Enzyme

3.2.1.113

PMID

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0050571	congenital disorder of glycosylation type II	Alliance of Genome Resources

The Human Phenotype Ontology

Displaying entries **11 - 20** of 35 in total

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HPO ID	HPO Term
HP:0000391	Thickened helices
HP:0000411	Protruding ear
HP:0000414	Bulbous nose
HP:0000430	Underdeveloped nasal alae
HP:0000431	Wide nasal bridge
HP:0000483	Astigmatism
HP:0000486	Strabismus
HP:0000490	Deeply set eye
HP:0000494	Downslanted palpebral fissures
HP:0000508	Ptosis

Displaying 1 entry
Disease ID	Disease Name
OMIM:619493	congenital disorder of glycosylation, type 2v

PubChem Disease

MedGen Diseases

Congenital disorder of glycosylation, type 2v

OMIM Phenotypes

CONGENITAL DISORDER OF GLYCOSYLATION, TYPE 2v; CDG2V

KEGG Diseases

Congenital disorders of glycosylation type II

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

Ortholog

Displaying entries **1 - 10** of 96 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Xenopus tropicalis	34714	Xenbase:XB-GENE-5811499
Mus musculus	66967	Xenbase:XB-GENE-5811499	MOUSE27286
Xenopus tropicalis	80267	Xenbase:XB-GENE-5811499
Xenopus tropicalis	181210	Xenbase:XB-GENE-5811499
Rattus norvegicus	289085		RATNO07254
Xenopus tropicalis	443907	Xenbase:XB-GENE-5811499
Pan troglodytes	457578		PANTR23213
Canis lupus familiaris	480040		CANLF17182
Bos taurus	519058		BOVIN08779
Danio rerio	559811		DANRE43895

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

LM-GlycoRepo

All Resources

Other Information

Tools

Search Tools

Guidelines

Summary

External Links

Annotation

OrthoDB (Group)

KEGG BRITE Database

PubChem Disease

International Collaboration

Acknowledgements