Aliases for STT3B Gene
- STT3B, Catalytic Subunit Of The Oligosaccharyltransferase Complex 2 3 5
- Dolichyl-Diphosphooligosaccharide Protein Glycotransferase 2 3
- Source Of Immunodominant MHC-Associated Peptides Homolog 3 4
- Oligosaccharyl Transferase Subunit STT3B 3 4
- STT3-B 3 4
- SIMP 3 4
- STT3, Subunit Of The Oligosaccharyltransferase Complex, Homolog B (S. Cerevisiae) 2
- Dolichyl-Diphosphooligosaccharide--Protein Glycosyltransferase Subunit STT3B 3
The protein encoded by this gene is a catalytic subunit of a protein complex that transfers oligosaccharides onto asparagine residues. Defects in this gene are a cause of congenital disorder of glycosylation Ix (CDG1X). [provided by RefSeq, Jun 2014]
GeneCards Summary for STT3B Gene
STT3B (STT3B, Catalytic Subunit Of The Oligosaccharyltransferase Complex) is a Protein Coding gene. Diseases associated with STT3B include Congenital Disorder Of Glycosylation, Type Ix and Stt3b-Cdg. Among its related pathways are Protein processing in endoplasmic reticulum and Metabolism. GO annotations related to this gene include dolichyl-diphosphooligosaccharide-protein glycotransferase activity and oligosaccharyl transferase activity. An important paralog of this gene is STT3A.
UniProtKB/Swiss-Prot for STT3B Gene
Catalytic 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. 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). STT3B is present in a small subset of OST complexes and mediates both cotranslational and post-translational N-glycosylation of target proteins: STT3B-containing complexes are required for efficient post-translational glycosylation and while they are less competent than STT3A-containing complexes for cotranslational glycosylation, they have the ability to mediate glycosylation of some nascent sites that are not accessible for STT3A. STT3B-containing complexes also act post-translationally and mediate modification of skipped glycosylation sites in unfolded proteins. Plays a role in ER-associated degradation (ERAD) pathway that mediates ubiquitin-dependent degradation of misfolded endoplasmic reticulum proteins by mediating N-glycosylation of unfolded proteins, which are then recognized by the ERAD pathway and targeted for degradation. Mediates glycosylation of the disease variant AMYL-TTR Asp-38 of TTR at Asn-118, leading to its degradation.