DDOST

This gene encodes a component of the oligosaccharyltransferase complex which catalyzes the transfer of high-mannose oligosaccharides to asparagine residues on nascent polypeptides in the lumen of the rough endoplasmic reticulum. The protein complex co-purifies with ribosomes. The product of this gene is also implicated in the processing of advanced glycation endproducts (AGEs), which form from non-enzymatic reactions between sugars and proteins or lipids and are associated with aging and hyperglycemia. [provided by RefSeq]

Full Name

dolichyl-diphosphooligosaccharide-protein glycosyltransferase

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. Required for the assembly of both SST3A- and SS3B-containing OST complexes. Required for efficient N-glycosylation.

Subunit of the oligosaccharyl transferase (OST) complex that catalyzes the initial transfer of a defined glycan (Glc3Man9GlcNAc2 in eukaryotes) from the lipid carrier dolichol-pyrophosphate to an asparagine residue within an Asn-X-Ser/Thr consensus motif in nascent polypeptide chains, the first step in protein N-glycosylation. N-glycosylation occurs cotranslationally and the complex associates with the Sec61 complex at the channel-forming translocon complex that mediates protein translocation across the endoplasmic reticulum (ER). All subunits are required for a maximal enzyme activity (By similarity).

Required for the assembly of both SST3A- and SS3B-containing OST complexes (PubMed:22467853).

Biological Process

Neutrophil degranulation Source: Reactome
Protein glycosylation Source: UniProtKB
Protein N-linked glycosylation Source: UniProtKB
Protein N-linked glycosylation via asparagine Source: GO_Central
Regulation of protein stability Source: ARUK-UCL
Response to cytokine Source: UniProtKB
T cell activation Source: UniProtKB
Viral protein processing Source: Reactome

Cellular Location

Endoplasmic reticulum membrane

Involvement in disease

Congenital disorder of glycosylation 1R (CDG1R):
A form of congenital disorder of glycosylation, 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.

Topology

Lumenal: 43-427
Helical: 428-447
Cytoplasmic: 448-456