Gene/Proteome Database (LMPD)
LMPD ID
LMP006110
Gene ID
Species
Homo sapiens (Human)
Gene Name
dolichyl-phosphate mannosyltransferase polypeptide 3
Gene Symbol
Synonyms
CDG1O
Chromosome
1
Map Location
1q22
Summary
Dolichol-phosphate mannose (Dol-P-Man) serves as a donor of mannosyl residues on the lumenal side of the endoplasmic reticulum (ER). Lack of Dol-P-Man results in defective surface expression of GPI-anchored proteins. Dol-P-Man is synthesized from GDP-mannose and dolichol-phosphate on the cytosolic side of the ER by the enzyme dolichyl-phosphate mannosyltransferase. The protein encoded by this gene is a subunit of dolichyl-phosphate mannosyltransferase and acts as a stabilizer subunit of the dolichyl-phosphate mannosyltransferase complex. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
Gene Information
Entrez Gene ID
Gene Name
dolichyl-phosphate mannosyltransferase polypeptide 3
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0033185 | IDA:UniProtKB | C | dolichol-phosphate-mannose synthase complex |
| GO:0005783 | IDA:UniProtKB | C | endoplasmic reticulum |
| GO:0005789 | TAS:HGNC | C | endoplasmic reticulum membrane |
| GO:0030176 | IDA:HGNC | C | integral component of endoplasmic reticulum membrane |
| GO:0031501 | TAS:HGNC | C | mannosyltransferase complex |
| GO:0016020 | IDA:UniProtKB | C | membrane |
| GO:0006501 | TAS:Reactome | P | C-terminal protein lipidation |
| GO:0006506 | IDA:UniProtKB | P | GPI anchor biosynthetic process |
| GO:0005975 | NAS:ProtInc | P | carbohydrate metabolic process |
| GO:0044267 | TAS:Reactome | P | cellular protein metabolic process |
| GO:0006488 | TAS:Reactome | P | dolichol-linked oligosaccharide biosynthetic process |
| GO:0043687 | TAS:Reactome | P | post-translational protein modification |
| GO:0018406 | TAS:HGNC | P | protein C-linked glycosylation via 2'-alpha-mannosyl-L-tryptophan |
| GO:0018279 | TAS:Reactome | P | protein N-linked glycosylation via asparagine |
| GO:0035269 | TAS:HGNC | P | protein O-linked mannosylation |
| GO:0035268 | TAS:HGNC | P | protein mannosylation |
| GO:0031647 | IPI:UniProtKB | P | regulation of protein stability |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| hsa01100 | Metabolic pathways |
| hsa00510 | N-Glycan biosynthesis |
| ko00510 | N-Glycan biosynthesis |
BIOCYC Pathway Links
| BIOCYC Pathway ID | Description |
|---|---|
| MANNOSYL-CHITO-DOLICHOL-BIOSYNTHESIS | dolichyl-diphosphooligosaccharide biosynthesis |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_22426 | Asparagine N-linked glycosylation |
| REACT_22433 | Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein |
| REACT_17015 | Metabolism of proteins |
| REACT_1853 | Post-translational modification: synthesis of GPI-anchored proteins |
| REACT_22161 | Post-translational protein modification |
| REACT_2032 | Synthesis of dolichyl-phosphate mannose |
| REACT_22387 | Synthesis of substrates in N-glycan biosythesis |
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR013174 | Dolichol-phosphate mannosyltransferase subunit 3 |
UniProt Annotations
Entry Information
Gene Name
dolichyl-phosphate mannosyltransferase polypeptide 3
Protein Entry
DPM3_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative splicing; Named isoforms=2; Name=1; Synonyms=Short; IsoId=Q9P2X0-1; Sequence=Displayed; Name=2; Synonyms=Long; IsoId=Q9P2X0-2; Sequence=VSP_001308; |
| Disease | Congenital disorder of glycosylation 1O (CDG1O) [MIM:612937]: 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. CDG1O patients have increased serum creatine kinase, dystrophic changes on muscle biopsy, and reduced O-mannosylation of alpha-dystroglycan. {ECO:0000269|PubMed:19576565}. Note=The disease is caused by mutations affecting the gene represented in this entry. |
| Function | Stabilizer subunit of the dolichol-phosphate mannose (DPM) synthase complex; tethers catalytic subunit DPM1 to the ER. {ECO:0000269|PubMed:10835346}. |
| Interaction | O60762:DPM1; NbExp=4; IntAct=EBI-9087337, EBI-719526; Q9Z325:Dpm2 (xeno); NbExp=2; IntAct=EBI-9087337, EBI-9097185; |
| Pathway | Protein modification; protein glycosylation. |
| Similarity | Belongs to the DPM3 family. {ECO:0000305}. |
| Subcellular Location | Endoplasmic reticulum membrane; Multi-pass membrane protein. |
| Subunit | Component of the dolichol-phosphate mannose (DPM) synthase complex composed of DPM1, DPM2 and DPM3; in the complex associated with DPM1 via its C-terminal domain and with DPM2 via its N-terminal portion. {ECO:0000269|PubMed:10835346}. |
| Web Resource | Name=GGDB; Note=GlycoGene database; URL="http://jcggdb.jp/rcmg/ggdb/Homolog?cat=symbol&symbol=DPM3"; |
Identical and Related Proteins
Unique RefSeq proteins for LMP006110 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 19424120 | RefSeq | NP_061846 | 122 | dolichol-phosphate mannosyltransferase subunit 3 isoform 1 |
| 24430135 | RefSeq | NP_714963 | 92 | dolichol-phosphate mannosyltransferase subunit 3 isoform 2 |
Identical Sequences to LMP006110 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:19424120 | GenBank | EAW53126.1 | 122 | dolichyl-phosphate mannosyltransferase polypeptide 3, isoform CRA_b [Homo sapiens] |
| GI:19424120 | GenBank | AEH67659.1 | 122 | Sequence 45 from patent US 7928215 |
| GI:24430135 | GenBank | AFO15177.1 | 92 | Sequence 661 from patent US 8211634 |
| GI:19424120 | GenBank | AFQ84268.1 | 122 | Sequence 45 from patent US 8252294 |
| GI:19424120 | GenBank | JAA01656.1 | 122 | dolichyl-phosphate mannosyltransferase polypeptide 3 [Pan troglodytes] |
| GI:19424120 | GenBank | JAA33594.1 | 122 | dolichyl-phosphate mannosyltransferase polypeptide 3 [Pan troglodytes] |
| GI:19424120 | GenBank | AIC51599.1 | 122 | DPM3, partial [synthetic construct] |
| GI:24430135 | RefSeq | XP_003308482.1 | 92 | PREDICTED: dolichol-phosphate mannosyltransferase subunit 3 [Pan troglodytes] |
| GI:24430135 | RefSeq | XP_003817119.1 | 92 | PREDICTED: dolichol-phosphate mannosyltransferase subunit 3 [Pan paniscus] |
| GI:24430135 | RefSeq | XP_004062364.1 | 92 | PREDICTED: dolichol-phosphate mannosyltransferase subunit 3-like isoform 1 [Gorilla gorilla gorilla] |
| GI:24430135 | RefSeq | XP_004062365.1 | 92 | PREDICTED: dolichol-phosphate mannosyltransferase subunit 3-like isoform 2 [Gorilla gorilla gorilla] |
| GI:24430135 | RefSeq | XP_008968490.1 | 92 | PREDICTED: dolichol-phosphate mannosyltransferase subunit 3 [Pan paniscus] |
Related Sequences to LMP006110 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:19424120 | DBBJ | BAG57082.1 | 168 | unnamed protein product [Homo sapiens] |
| GI:24430135 | GenBank | AAK28487.1 | 122 | prostin 1 long isoform [Homo sapiens] |
| GI:19424120 | GenBank | AAH32223.2 | 94 | DPM3 protein, partial [Homo sapiens] |
| GI:19424120 | GenBank | AAH65233.1 | 106 | DPM3 protein, partial [Homo sapiens] |
| GI:24430135 | GenBank | AAI04204.1 | 122 | Dolichyl-phosphate mannosyltransferase polypeptide 3 [Homo sapiens] |
| GI:19424120 | GenBank | AAI04482.1 | 113 | DPM3 protein, partial [Homo sapiens] |
| GI:24430135 | GenBank | AFQ84268.1 | 122 | Sequence 45 from patent US 8252294 |
| GI:24430135 | GenBank | JAA01656.1 | 122 | dolichyl-phosphate mannosyltransferase polypeptide 3 [Pan troglodytes] |
| GI:24430135 | GenBank | JAA33594.1 | 122 | dolichyl-phosphate mannosyltransferase polypeptide 3 [Pan troglodytes] |
| GI:24430135 | RefSeq | NP_061846.2 | 122 | dolichol-phosphate mannosyltransferase subunit 3 isoform 1 [Homo sapiens] |
| GI:19424120 | RefSeq | XP_003892808.1 | 109 | PREDICTED: dolichol-phosphate mannosyltransferase subunit 3 isoform X1 [Papio anubis] |
| GI:19424120 | RefSeq | XP_004062365.1 | 92 | PREDICTED: dolichol-phosphate mannosyltransferase subunit 3-like isoform 2 [Gorilla gorilla gorilla] |