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Protein

Putative Dol-P-Glc:Glc(2)Man(9)GlcNAc(2)-PP-Dol alpha-1,2-glucosyltransferase

Gene

ALG10B

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Putative alpha-1,2-glucosyltransferase, which adds the third glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation. Transfers glucose from dolichyl phosphate glucose (Dol-P-Glc) onto the lipid-linked oligosaccharide Glc2Man9GlcNAc(2)-PP-Dol (By similarity). When coupled to KCNH2 may reduce KCNH2 sensitivity to classic proarrhythmic drug blockade, possibly by mediating glycosylation of KCNH2 (PubMed:14525949). Has a role in maintenance of cochlear outer hair cell function (By similarity).By similarity1 Publication

Catalytic activityi

Dolichyl beta-D-glucosyl phosphate + D-Glc-alpha-(1->3)-D-Glc-alpha-(1->3)-D-Man-alpha-(1->2)-D-Man-alpha-(1->2)-D-Man-alpha-(1->3)-[D-Man-alpha-(1->2)-D-Man-alpha-(1->3)-(D-Man-alpha-(1->2)-D-Man-alpha-(1->6))-D-Man-alpha-(1->6)]-D-Man-beta-(1->4)-D-GlcNAc-beta-(1->4)-D-GlcNAc-diphosphodolichol = D-Glc-alpha-(1->2)-D-Glc-alpha-(1->3)-D-Glc-alpha-(1->3)-D-Man-alpha-(1->2)-D-Man-alpha-(1->2)-D-Man-alpha-(1->3)-[D-Man-alpha-(1->2)-D-Man-alpha-(1->3)-(D-Man-alpha-(1->2)-D-Man-alpha-(1->6))-D-Man-alpha-(1->6)]-D-Man-beta-(1->4)-D-GlcNAc-beta-(1->4)-D-GlcNAc-diphosphodolichol + dolichyl phosphate.By similarity

Pathwayi: protein glycosylation

This protein is involved in the pathway protein glycosylation, which is part of Protein modification.
View all proteins of this organism that are known to be involved in the pathway protein glycosylation and in Protein modification.

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Glycosyltransferase, Transferase

Enzyme and pathway databases

ReactomeiR-HSA-446193. Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein.
UniPathwayiUPA00378.

Names & Taxonomyi

Protein namesi
Recommended name:
Putative Dol-P-Glc:Glc(2)Man(9)GlcNAc(2)-PP-Dol alpha-1,2-glucosyltransferaseBy similarity (EC:2.4.1.256By similarity)
Alternative name(s):
Alpha-1,2-glucosyltransferase ALG10-A
Alpha-2-glucosyltransferase ALG10-B
Asparagine-linked glycosylation protein 10 homolog B
Potassium channel regulator 1
Gene namesi
Name:ALG10B
Synonyms:KCR1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

HGNCiHGNC:31088. ALG10B.

Subcellular locationi

  • Cell membrane By similarity; Multi-pass membrane protein By similarity

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 6CytoplasmicSequence analysis6
Transmembranei7 – 27HelicalSequence analysisAdd BLAST21
Topological domaini28 – 64ExtracellularSequence analysisAdd BLAST37
Transmembranei65 – 85HelicalSequence analysisAdd BLAST21
Topological domaini86 – 97CytoplasmicSequence analysisAdd BLAST12
Transmembranei98 – 118HelicalSequence analysisAdd BLAST21
Topological domaini119 – 126ExtracellularSequence analysis8
Transmembranei127 – 147HelicalSequence analysisAdd BLAST21
Topological domaini148 – 150CytoplasmicSequence analysis3
Transmembranei151 – 171HelicalSequence analysisAdd BLAST21
Topological domaini172 – 175ExtracellularSequence analysis4
Transmembranei176 – 196HelicalSequence analysisAdd BLAST21
Topological domaini197 – 256CytoplasmicSequence analysisAdd BLAST60
Transmembranei257 – 277HelicalSequence analysisAdd BLAST21
Topological domaini278 – 283ExtracellularSequence analysis6
Transmembranei284 – 304HelicalSequence analysisAdd BLAST21
Topological domaini305 – 317CytoplasmicSequence analysisAdd BLAST13
Transmembranei318 – 338HelicalSequence analysisAdd BLAST21
Topological domaini339 – 365ExtracellularSequence analysisAdd BLAST27
Transmembranei366 – 386HelicalSequence analysisAdd BLAST21
Topological domaini387 – 392CytoplasmicSequence analysis6
Transmembranei393 – 413HelicalSequence analysisAdd BLAST21
Topological domaini414 – 436ExtracellularSequence analysisAdd BLAST23
Transmembranei437 – 457HelicalSequence analysisAdd BLAST21
Topological domaini458 – 473CytoplasmicSequence analysisAdd BLAST16

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cell membrane, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi144245.
OpenTargetsiENSG00000175548.
PharmGKBiPA134936082.

Polymorphism and mutation databases

BioMutaiALG10B.
DMDMi296434391.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002154481 – 473Putative Dol-P-Glc:Glc(2)Man(9)GlcNAc(2)-PP-Dol alpha-1,2-glucosyltransferaseAdd BLAST473

Proteomic databases

EPDiQ5I7T1.
MaxQBiQ5I7T1.
PaxDbiQ5I7T1.
PeptideAtlasiQ5I7T1.
PRIDEiQ5I7T1.

PTM databases

iPTMnetiQ5I7T1.
PhosphoSitePlusiQ5I7T1.

Expressioni

Tissue specificityi

Highly expressed in heart, placenta, liver, kidney and pancreas. Weakly expressed in lung, skeletal muscle and brain.1 Publication

Gene expression databases

BgeeiENSG00000175548.
CleanExiHS_ALG10B.
ExpressionAtlasiQ5I7T1. baseline and differential.
GenevisibleiQ5I7T1. HS.

Organism-specific databases

HPAiHPA043329.
HPA063387.

Interactioni

Subunit structurei

Interacts with KCNH1 and KCNH2.By similarity

Protein-protein interaction databases

BioGridi126840. 4 interactors.
STRINGi9606.ENSP00000310120.

Structurei

3D structure databases

ProteinModelPortaliQ5I7T1.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the ALG10 glucosyltransferase family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG2642. Eukaryota.
ENOG410YGXE. LUCA.
GeneTreeiENSGT00390000012906.
HOGENOMiHOG000240902.
HOVERGENiHBG053222.
InParanoidiQ5I7T1.
KOiK03850.
OMAiHTRKGFA.
OrthoDBiEOG091G0A27.
PhylomeDBiQ5I7T1.
TreeFamiTF300150.

Family and domain databases

InterProiIPR016900. Alg10.
[Graphical view]
PANTHERiPTHR12989. PTHR12989. 1 hit.
PfamiPF04922. DIE2_ALG10. 1 hit.
[Graphical view]
PIRSFiPIRSF028810. Alpha1_2_glucosyltferase_Alg10. 1 hit.

Sequencei

Sequence statusi: Complete.

Q5I7T1-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAQLEGYCFS AALSCTFLVS CLLFSAFSRA LREPYMDEIF HLPQAQRYCE
60 70 80 90 100
GHFSLSQWDP MITTLPGLYL VSVGVVKPAI WIFAWSEHVV CSIGMLRFVN
110 120 130 140 150
LLFSVGNFYL LYLLFHKVQP RNKAASSIQR VLSTLTLAVF PTLYFFNFLY
160 170 180 190 200
YTEAGSMFFT LFAYLMCLYG NHKTSAFLGF CGFMFRQTNI IWAVFCAGNV
210 220 230 240 250
IAQKLTEAWK TELQKKEDRL PPIKGPFAEF RKILQFLLAY SMSFKNLSML
260 270 280 290 300
FCLTWPYILL GFLFCAFVVV NGGIVIGDRS SHEACLHFPQ LFYFFSFTLF
310 320 330 340 350
FSFPHLLSPS KIKTFLSLVW KHGILFLVVT LVSVFLVWKF TYAHKYLLAD
360 370 380 390 400
NRHYTFYVWK RVFQRYAILK YLLVPAYIFA GWSIADSLKS KPIFWNLMFF
410 420 430 440 450
ICLFIVIVPQ KLLEFRYFIL PYVIYRLNIT LPPTSRLVCE LSCYAIVNFI
460 470
TFYIFLNKTF QWPNSQDIQR FMW
Length:473
Mass (Da):55,448
Last modified:May 18, 2010 - v2
Checksum:i47D0CF7B6C84B8EC
GO

Polymorphismi

Genetic variations in ALG10B may reduce susceptibility to acquired long QT syndrome.

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04821784A → G.3 PublicationsCorresponds to variant rs6582584dbSNPEnsembl.1
Natural variantiVAR_061002383S → N.Corresponds to variant rs57963306dbSNPEnsembl.1
Natural variantiVAR_023753446I → V in some patients with drug-induced cardiac repolarization defects; exerts a greater protective effect, relative to wild-type, against drug blockage of KCNH2 potassium channel. 1 PublicationCorresponds to variant rs61730283dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY845858 mRNA. Translation: AAW31756.1.
AC117372 Genomic DNA. No translation available.
CH471111 Genomic DNA. Translation: EAW57795.1.
BC137413 mRNA. Translation: AAI37414.1.
BC137414 mRNA. Translation: AAI37415.1.
CCDSiCCDS31772.1.
RefSeqiNP_001013642.1. NM_001013620.3.
UniGeneiHs.259305.

Genome annotation databases

EnsembliENST00000308742; ENSP00000310120; ENSG00000175548.
GeneIDi144245.
KEGGihsa:144245.
UCSCiuc001rln.5. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY845858 mRNA. Translation: AAW31756.1.
AC117372 Genomic DNA. No translation available.
CH471111 Genomic DNA. Translation: EAW57795.1.
BC137413 mRNA. Translation: AAI37414.1.
BC137414 mRNA. Translation: AAI37415.1.
CCDSiCCDS31772.1.
RefSeqiNP_001013642.1. NM_001013620.3.
UniGeneiHs.259305.

3D structure databases

ProteinModelPortaliQ5I7T1.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi126840. 4 interactors.
STRINGi9606.ENSP00000310120.

PTM databases

iPTMnetiQ5I7T1.
PhosphoSitePlusiQ5I7T1.

Polymorphism and mutation databases

BioMutaiALG10B.
DMDMi296434391.

Proteomic databases

EPDiQ5I7T1.
MaxQBiQ5I7T1.
PaxDbiQ5I7T1.
PeptideAtlasiQ5I7T1.
PRIDEiQ5I7T1.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000308742; ENSP00000310120; ENSG00000175548.
GeneIDi144245.
KEGGihsa:144245.
UCSCiuc001rln.5. human.

Organism-specific databases

CTDi144245.
DisGeNETi144245.
GeneCardsiALG10B.
HGNCiHGNC:31088. ALG10B.
HPAiHPA043329.
HPA063387.
MIMi603313. gene.
neXtProtiNX_Q5I7T1.
OpenTargetsiENSG00000175548.
PharmGKBiPA134936082.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2642. Eukaryota.
ENOG410YGXE. LUCA.
GeneTreeiENSGT00390000012906.
HOGENOMiHOG000240902.
HOVERGENiHBG053222.
InParanoidiQ5I7T1.
KOiK03850.
OMAiHTRKGFA.
OrthoDBiEOG091G0A27.
PhylomeDBiQ5I7T1.
TreeFamiTF300150.

Enzyme and pathway databases

UniPathwayiUPA00378.
ReactomeiR-HSA-446193. Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein.

Miscellaneous databases

ChiTaRSiALG10B. human.
GenomeRNAii144245.
PROiQ5I7T1.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000175548.
CleanExiHS_ALG10B.
ExpressionAtlasiQ5I7T1. baseline and differential.
GenevisibleiQ5I7T1. HS.

Family and domain databases

InterProiIPR016900. Alg10.
[Graphical view]
PANTHERiPTHR12989. PTHR12989. 1 hit.
PfamiPF04922. DIE2_ALG10. 1 hit.
[Graphical view]
PIRSFiPIRSF028810. Alpha1_2_glucosyltferase_Alg10. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiAG10B_HUMAN
AccessioniPrimary (citable) accession number: Q5I7T1
Secondary accession number(s): B2RPF4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 8, 2005
Last sequence update: May 18, 2010
Last modified: November 2, 2016
This is version 105 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Caution

PubMed:15280551 reports a Val-447 variant, however there is evidence that the right variant is Val-446.Curated

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.