InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: MOGS

Summary

InnateDB Protein

IDBP-57449.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

MOGS

Protein Name

mannosyl-oligosaccharide glucosidase

Synonyms

CDG2B; CWH41; DER7; GCS1;

Species

Homo sapiens

Ensembl Protein

ENSP00000233616

InnateDB Gene

IDBG-57447 (MOGS)

Protein Structure

UniProt Annotation

Function

Cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner.

Subcellular Localization

Endoplasmic reticulum membrane; Single-pass type II membrane protein.

Disease Associations

Type IIb congenital disorder of glycosylation (CDGIIb) [MIM:606056]: Characterized 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. {ECO:0000269PubMed:10788335}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 24 experimentally validated interaction(s) in this database.

Experimentally validated
Total	24 [view]
Protein-Protein	24 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0004573	mannosyl-oligosaccharide glucosidase activity
GO:0015926	glucosidase activity

Biological Process

GO:0006457	protein folding
GO:0006487	protein N-linked glycosylation
GO:0009311	oligosaccharide metabolic process
GO:0018279	protein N-linked glycosylation via asparagine
GO:0043687	post-translational protein modification
GO:0044267	cellular protein metabolic process

Cellular Component

GO:0005783	endoplasmic reticulum
GO:0005789	endoplasmic reticulum membrane
GO:0016020	membrane
GO:0016021	integral component of membrane
GO:0070062	extracellular vesicular exosome