InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: DDOST

Summary

InnateDB Protein

IDBP-92936.5

Last Modified

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

Gene Symbol

DDOST

Protein Name

dolichyl-diphosphooligosaccharide--protein glycosyltransferase

Synonyms

AGER1; CDG1R; OKSWcl45; OST; OST48; WBP1;

Species

Homo sapiens

Ensembl Protein

ENSP00000364188

InnateDB Gene

IDBG-409236 (DDOST)

Protein Structure

UniProt Annotation

Function

Essential subunit of the N-oligosaccharyl transferase (OST) complex which catalyzes the transfer of a high mannose oligosaccharide from a lipid-linked oligosaccharide donor to an asparagine residue within an Asn-X-Ser/Thr consensus motif in nascent polypeptide chains.

Subcellular Localization

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

Disease Associations

Congenital disorder of glycosylation 1R (CDG1R) [MIM:614507]: A multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N- glycoproteins during embryonic development, differentiation, and maintenance of cell functions. {ECO:0000269PubMed:22305527}. 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 52 experimentally validated interaction(s) in this database.

Experimentally validated
Total	52 [view]
Protein-Protein	51 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004576	oligosaccharyl transferase activity
GO:0004579	dolichyl-diphosphooligosaccharide-protein glycotransferase activity
GO:0005515	protein binding

Biological Process

GO:0006412	translation
GO:0006486	protein glycosylation
GO:0006487	protein N-linked glycosylation
GO:0006614	SRP-dependent cotranslational protein targeting to membrane
GO:0010467	gene expression
GO:0018279	protein N-linked glycosylation via asparagine
GO:0034097	response to cytokine
GO:0042110	T cell activation
GO:0043687	post-translational protein modification
GO:0044267	cellular protein metabolic process
GO:0045087	innate immune response

Cellular Component

GO:0005783	endoplasmic reticulum
GO:0005789	endoplasmic reticulum membrane
GO:0008250	oligosaccharyltransferase complex
GO:0016020	membrane
GO:0016021	integral component of membrane
GO:0043231	intracellular membrane-bounded organelle
GO:0043234	protein complex