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 **1 - 10** of 35 in total

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000034	Hydrocele testis
HP:0000175	Cleft palate
HP:0000218	High palate
HP:0000219	Thin upper lip vermilion
HP:0000278	Retrognathia
HP:0000286	Epicanthus
HP:0000316	Hypertelorism
HP:0000322	Short philtrum
HP:0000369	Low-set ears

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 **21 - 30** of 96 in total

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Species	Gene ID	Orthologous MAtrix
Loxodonta africana	100668448	LOXAF13670
Oreochromis niloticus	100698159	ORENI42601
Sarcophilus harrisii	100925184	SARHA12548
Otolemur garnettii	100947853	OTOGA15358
Pan paniscus	100991162	PANPA03327
Papio anubis	101002411	PAPAN20538
Saimiri boliviensis boliviensis	101030824	SAIBB09018
Takifugu rubripes	101079995	TAKRU27218
Felis catus	101087192	FELCA37105
Ovis aries	101107978	SHEEP03972

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