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DDOST - dolichyl-diphosphooligosaccharide--protein glycosyltransferase non-catalytic subunit Gene

Also Known as OST; WBP1; AGER1; CDG1R; GATD6; OST48; OKSWcl45

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 1650

About DDOST

Cytogenetic location: 1p36.12 Genomic coordinates (GRCh38): 1:20,651,777-20,661,369 (from NCBI)

This gene has 5 transcripts (splice variants), 204 orthologues and is associated with 3 phenotypes. Ubiquitous expression in thyroid (RPKM 112.6), placenta (RPKM 100.3) and 25 other tissues.

Summary

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, Jul 2008]

DDOST Products (1)

mRNA	Protein	Name
NM_005216.5	NP_005207.3	dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables enzyme activator activity	IMP IMP: Inferred from mutant phenotype	22467853	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	21903422	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in T cell activation	IDA IDA: Inferred from direct assay	9642163	GOA
involved in protein N-linked glycosylation	IDA IDA: Inferred from direct assay	9642163	GOA
involved in protein N-linked glycosylation via asparagine	IMP IMP: Inferred from mutant phenotype	22467853	GOA
involved in regulation of protein stability	IMP IMP: Inferred from mutant phenotype	22467853	GOA
involved in response to cytokine	IDA IDA: Inferred from direct assay	9642163	GOA

Cellular Component GO Annotation	Evidence	References	Source
located in intracellular membrane-bounded organelle	IDA IDA: Inferred from direct assay	9642163	GOA
part of oligosaccharyltransferase complex	IDA IDA: Inferred from direct assay	9642163	GOA
part of oligosaccharyltransferase complex	IPI IPI: Inferred from physical interaction	31831667	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

DDOST Protein Structure

DDOST_48kD

0
100
200
300
400
456 a.a.

Protein Preferred Names	Protein Names
dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit	advanced glycation end-product receptor 1

Recombinant DDOST Proteins

Cat. No.	Product Name	Accession	Purity
HY-P75701	DDOST Protein, Human	P39656-1 (S43-P427)	≥ 95%, as determined by reducing SDS-PAGE.

Related Diseases

Diseases	Alias
Congenital Disorder Of Glycosylation, Type Ir	Congenital Disorder Of Glycosylation Type Ir CDG1R Cdg Ir Cdgir Congenital Disorder Of Glycosylation 1r Cdg-Ir Congenital Disorder Of Glycosylation Ir Ddost-Cdg Cdg Syndrome Type Ir Carbohydrate Deficient Glycoprotein Syndrome Type Ir Congenital Disorder Of Glycosylation Type 1r Glycosylation, Congenital Disorder Of, Type Ir
Congenital Disorder Of Glycosylation, Type In	Congenital Disorder Of Glycosylation CDG1N Congenital Disorders Of Glycosylation Cdg In Cdgin Congenital Disorder Of Glycosylation 1n Carbohydrate-Deficient Glycoprotein Syndrome Cdg Rft1-Cdg Cdg-In Congenital Disorder Of Glycosylation Type In Carbohydrate Deficient Glycoprotein Syndrome Cdg Syndrome Congenital Disorder Of Glycosylation In Carbohydrate-Deficient Glycoprotein Syndromes Cdg Syndrome Type In Carbohydrate Deficient Glycoprotein Syndrome Type In Congenital Disorder Of Glycosylation Type 1n Man5glcnac2-Pp-Dol Flippase Deficiency Glycosylation, Congenital Disorder Of Glycosylation, Congenital Disorder Of, Type In
Granulomatous Disease, Chronic, Autosomal Recessive, 2	Chronic Granulomatous Disease Due To Deficiency Of Ncf-2 CGD2 Ncf2 Deficiency P67-Phox Deficiency Autosomal Recessive Chronic Granulomatous Disease Cytochrome B-Positive Type Ii Granulomatous Disease, Chronic, Autosomal Recessive, Cytochrome B-Positive, Type 2 Granulomatous Disease, Chronic, Autosomal Recessive, Cytochrome B-Positive, Type Ii Cgd, Autosomal Recessive Cytochrome B-Positive, Type Ii Granulomatous Disease, Chronic, Due To Ncf2 Deficiency Neutrophil Cytosol Factor 2 Deficiency Chronic Granulomatous Disease 2, Autosomal Recessive Autosomal Recessive Chronic Granulomatous Disease 2 Cdg2 Deficiency Of Ncf2 Deficiency Of P67-Phox Chronic Granulomatous Disease Autosomal Recessive Cytochrome B-Positive Type Ii Deficiency Of Neutrophil Cytosol Factor 2 Avellino Corneal Dystrophy
Immunodeficiency 47	Congenital Disorder Of Glycosylation Type Ii CDG2E Congenital Disorder Of Glycosylation Type Iie IMD47 Cdg2s Cdg Iis Cdgiis Immunodeficiency And Hepatopathy With Or Without Neurologic Features Congenital Disorder Of Glycosylation, Type Ii CDG1I Congenital Disorder Of Glycosylation, Type Iie Cdg Iie Congenital Disorder Of Glycosylation Type 2e Congenital Disorder Of Glycosylation, Type Iis Cdg Ii Cdgii Cdgiie Carbohydrate Deficient Glycoprotein Syndrome Type Iie Cdg Syndrome Type Iie Congenital Disorder Of Glycosylation Ii Congenital Disorder Of Glycosylation 1i Cdg-Iie Alg2-Cdg Cdg-Ii Glycosylation, Congenital Disorder Of, Type Ii Cdgiide Congenital Disorder Of Glycosylation Type Iis Cog7-Cdg Cdg Syndrome Type Ii Carbohydrate Deficient Glycoprotein Syndrome Type Ii Congenital Disorder Of Glycosylation Type 1i Mannosyltransferase 2 Deficiency Congenital Disorder Of Glycosylation 2e Congenital Disorder Of Glycosylation 2s Congenital Disorders Of Glycosylation Type Ii Glycosylation, Congenital Disorder Of, Type Iie Immunodeficiency, Type 47 Congenital Disorder Of Glycosylation Type 2a
Autosomal Recessive Intellectual Developmental Disorder	Mental Retardation, Autosomal Recessive Autosomal Recessive Mental Retardation Autosomal Recessive Non-Syndromic Mental Retardation Autosomal Recessive Non-Syndromic Intellectual Disability

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (3)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS03619	DDOST Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS20390	Ddost Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS26899	Ddost Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Mus musculus	DDOST	MGD	MGI:1194508
Macaca mulatta	DDOST	VGNC	VGNC:71757
Bos taurus	DDOST	VGNC	VGNC:27952
Felis catus	DDOST	VGNC	VGNC:61394
Rattus norvegicus	DDOST	RGD	RGD:1308970
Canis familiaris	DDOST	VGNC	VGNC:39841
Others	DDOST	NCBI