Gene/Proteome Database (LMPD)

LMPD ID
LMP005763
Gene ID
56052
Species
Homo sapiens (Human)
Gene Name
ALG1, chitobiosyldiphosphodolichol beta-mannosyltransferase
Gene Symbol
ALG1
Synonyms
CDG1K; HMAT1; HMT-1; HMT1; MT-1; Mat-1; hMat-1
Alternate Names
chitobiosyldiphosphodolichol beta-mannosyltransferase; mannosyltransferase-1; beta-1,4 mannosyltransferase; beta-1,4-mannosyltransferase; GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase; asparagine-linked glycosylation protein 1 homolog; GDP-mannose-dolichol diphosphochitobiose mannosyltransferase; asparagine-linked glycosylation 1, beta-1,4-mannosyltransferase homolog; asparagine-linked glycosylation 1 homolog (yeast, beta-1,4-mannosyltransferase)
Chromosome
16
Map Location
16p13.3
EC Number
2.4.1.142
Summary
The enzyme encoded by this gene catalyzes the first mannosylation step in the biosynthesis of lipid-linked oligosaccharides. This gene is mutated in congenital disorder of glycosylation type Ik. [provided by RefSeq, Dec 2008]
Orthologs
View orthologs and multiple alignments for ALG1

Proteins

chitobiosyldiphosphodolichol beta-mannosyltransferase
Refseq ID NP_061982
Protein GI 41350216
UniProt ID Q9BT22
mRNA ID NM_019109
Length 464
RefSeq Status REVIEWED
MAASCLVLLALCLLLPLLLLGGWKRWRRGRAARHVVAVVLGDVGRSPRMQYHALSLAMHGFSVTLLGFCNSKPHDELLQNNRIQIVGLTELQSLAVGPRVFQYGVKVVLQAMYLLWKLMWREPGAYIFLQNPPGLPSIAVCWFVGCLCGSKLVIDWHNYGYSIMGLVHGPNHPLVLLAKWYEKFFGRLSHLNLCVTNAMREDLADNWHIRAVTVYDKPASFFKETPLDLQHRLFMKLGSMHSPFRARSEPEDPVTERSAFTERDAGSGLVTRLRERPALLVSSTSWTEDEDFSILLAALEKFEQLTLDGHNLPSLVCVITGKGPLREYYSRLIHQKHFQHIQVCTPWLEAEDYPLLLGSADLGVCLHTSSSGLDLPMKVVDMFGCCLPVCAVNFKCLHELVKHEENGLVFEDSEELAAQLQMLFSNFPDPAGKLNQFRKNLRESQQLRWDESWVQTVLPLVMDT

Gene Information

Entrez Gene ID
56052
Gene Name
ALG1, chitobiosyldiphosphodolichol beta-mannosyltransferase
Gene Symbol
ALG1
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005783 TAS:ProtInc C endoplasmic reticulum
GO:0005789 TAS:Reactome C endoplasmic reticulum membrane
GO:0016021 IEA:UniProtKB-KW C integral component of membrane
GO:0016020 IDA:UniProtKB C membrane
GO:0004578 IEA:UniProtKB-EC F chitobiosyldiphosphodolichol beta-mannosyltransferase activity
GO:0000030 TAS:ProtInc F mannosyltransferase activity
GO:0044267 TAS:Reactome P cellular protein metabolic process
GO:0006488 TAS:Reactome P dolichol-linked oligosaccharide biosynthetic process
GO:0009103 TAS:ProtInc P lipopolysaccharide biosynthetic process
GO:0097502 TAS:GOC P mannosylation
GO:0043687 TAS:Reactome P post-translational protein modification
GO:0018279 TAS:Reactome P protein N-linked glycosylation via asparagine
GO:0006486 TAS:ProtInc P protein glycosylation

KEGG Pathway Links

KEGG Pathway ID Description
hsa01100 Metabolic pathways
hsa00510 N-Glycan biosynthesis
ko00510 N-Glycan biosynthesis

Domain Information

InterPro Annotations

Accession Description
IPR026051 Asparagine-linked glycosylation protein 1-like

UniProt Annotations

Entry Information

Gene Name
ALG1, chitobiosyldiphosphodolichol beta-mannosyltransferase
Protein Entry
ALG1_HUMAN
UniProt ID
Q9BT22
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9BT22-1; Sequence=Displayed; Name=2; IsoId=Q9BT22-2; Sequence=VSP_056931; Note=No experimental confirmation available;
Catalytic Activity GDP-mannose + chitobiosyldiphosphodolichol = GDP + beta-1,4-D-mannosylchitobiosyldiphosphodolichol.
Disease Congenital disorder of glycosylation 1K (CDG1K) [MIM
Function Participates in the formation of the lipid-linked precursor oligosaccharide for N-glycosylation. Involved in assembling the dolichol-pyrophosphate-GlcNAc(2)-Man(5) intermediate on the cytoplasmic surface of the ER.
Pathway Protein modification; protein glycosylation.
Sequence Caution Sequence=AAQ89432.1; Type=Erroneous initiation; Evidence= ;
Similarity Belongs to the glycosyltransferase group 1 family. Glycosyltransferase 33 subfamily.
Subcellular Location Endoplasmic reticulum membrane {ECO
Web Resource Name=GGDB; Note=GlycoGene database; URL="http://jcggdb.jp/rcmg/ggdb/Homolog?cat=symbol&symbol=ALG1";

Identical and Related Proteins

Unique RefSeq proteins for LMP005763 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
41350216 RefSeq NP_061982 464 chitobiosyldiphosphodolichol beta-mannosyltransferase

Identical Sequences to LMP005763 proteins

Reference Database Accession Length Protein Name
GI:41350216 DBBJ BAC11576.1 464 unnamed protein product [Homo sapiens]
GI:41350216 EMBL CAI72079.1 464 unnamed protein product [Homo sapiens]
GI:41350216 GenBank ABL23968.1 464 Sequence 54 from patent US 7129338
GI:41350216 GenBank EAW85240.1 464 asparagine-linked glycosylation 1 homolog (yeast, beta-1,4-mannosyltransferase), isoform CRA_a [Homo sapiens]
GI:41350216 GenBank AFG79906.1 464 Sequence 165 from patent US 8137928
GI:41350216 SwissProt Q9BT22.2 464 RecName: Full=Chitobiosyldiphosphodolichol beta-mannosyltransferase; AltName: Full=Asparagine-linked glycosylation protein 1 homolog; AltName: Full=Beta-1,4-mannosyltransferase; AltName: Full=GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase; AltName: Full=GDP-mannose-dolichol diphosphochitobiose mannosyltransferase; AltName: Full=Mannosyltransferase-1; Short=MT-1; Short=hMat-1 [Homo sapiens]

Related Sequences to LMP005763 proteins

Reference Database Accession Length Protein Name
GI:41350216 GenBank AAH04402.1 464 Asparagine-linked glycosylation 1, beta-1,4-mannosyltransferase homolog (S. cerevisiae) [Homo sapiens]
GI:41350216 GenBank AAH31095.1 464 Asparagine-linked glycosylation 1, beta-1,4-mannosyltransferase homolog (S. cerevisiae) [Homo sapiens]
GI:41350216 GenBank AAQ89432.1 472 mannosyltransferase, partial [Homo sapiens]
GI:41350216 GenBank ABM85936.1 464 asparagine-linked glycosylation 1 homolog (S. cerevisiae, beta-1,4-mannosyltransferase), partial [synthetic construct]
GI:41350216 GenBank ABW03760.1 464 asparagine-linked glycosylation 1 homolog (S. cerevisiae, beta-1,4-mannosyltransferase) [synthetic construct]
GI:41350216 GenBank AIC56772.1 464 ALG1, partial [synthetic construct]