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

Homo sapiens

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

Gene ID: 1650 | Gene type: protein coding

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	Reference	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	Reference	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	Reference	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%

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

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS03619	DDOST Human Pre-designed siRNA Set A	Pre-designed Sets	/	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

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