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Protein

Neutral alpha-glucosidase AB

Gene

GANAB

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

Functioni

Cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc2Man9GlcNAc2 oligosaccharide precursor of immature glycoproteins.1 Publication

Catalytic activityi

Hydrolysis of terminal (1->3)-alpha-D-glucosidic links in (1->3)-alpha-D-glucans.

Pathway: N-glycan metabolism

This protein is involved in the pathway N-glycan metabolism, which is part of Glycan metabolism.
View all proteins of this organism that are known to be involved in the pathway N-glycan metabolism and in Glycan metabolism.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Active sitei542 – 5421Nucleophile
Active sitei545 – 5451By similarity
Active sitei618 – 6181Proton donorBy similarity

GO - Molecular functioni

  • carbohydrate binding Source: InterPro
  • glucan 1,3-alpha-glucosidase activity Source: UniProtKB-EC
  • poly(A) RNA binding Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Glycosidase, Hydrolase

Enzyme and pathway databases

BRENDAi3.2.1.84. 2681.
ReactomeiREACT_23810. Calnexin/calreticulin cycle.
REACT_23878. N-glycan trimming in the ER and Calnexin/Calreticulin cycle.
UniPathwayiUPA00957.

Protein family/group databases

CAZyiGH31. Glycoside Hydrolase Family 31.

Names & Taxonomyi

Protein namesi
Recommended name:
Neutral alpha-glucosidase AB (EC:3.2.1.84)
Alternative name(s):
Alpha-glucosidase 2
Glucosidase II subunit alpha
Gene namesi
Name:GANAB
Synonyms:G2AN, KIAA0088
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 11

Organism-specific databases

HGNCiHGNC:4138. GANAB.

Subcellular locationi

GO - Cellular componenti

  • endoplasmic reticulum lumen Source: Reactome
  • extracellular exosome Source: UniProtKB
  • glucosidase II complex Source: Ensembl
  • Golgi apparatus Source: UniProtKB-SubCell
  • melanosome Source: UniProtKB-SubCell
  • membrane Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Endoplasmic reticulum, Golgi apparatus

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi542 – 5421D → N: Loss of activity. 1 Publication

Organism-specific databases

PharmGKBiPA28551.

Chemistry

DrugBankiDB00491. Miglitol.

Polymorphism and mutation databases

DMDMi54037162.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 2828Sequence AnalysisAdd
BLAST
Chaini29 – 944916Neutral alpha-glucosidase ABPRO_0000018571Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi97 – 971N-linked (GlcNAc...)1 Publication

Keywords - PTMi

Glycoprotein

Proteomic databases

MaxQBiQ14697.
PaxDbiQ14697.
PRIDEiQ14697.

2D gel databases

REPRODUCTION-2DPAGEIPI00383581.

PTM databases

PhosphoSiteiQ14697.

Expressioni

Tissue specificityi

Detected in placenta.1 Publication

Gene expression databases

BgeeiQ14697.
CleanExiHS_GANAB.
ExpressionAtlasiQ14697. baseline and differential.
GenevisibleiQ14697. HS.

Organism-specific databases

HPAiHPA026874.
HPA061426.

Interactioni

Subunit structurei

Heterodimer of a catalytic alpha subunit (GANAB) and a beta subunit (PRKCSH). Binds glycosylated PTPRC (By similarity).By similarity

Binary interactionsi

WithEntry#Exp.IntActNotes
ELF3P785451EBI-348004,EBI-1057285
PRRC2AP486341EBI-348004,EBI-347545
TRAF6Q9Y4K31EBI-348004,EBI-359276

Protein-protein interaction databases

BioGridi116802. 69 interactions.
IntActiQ14697. 17 interactions.
MINTiMINT-5001279.
STRINGi9606.ENSP00000340466.

Structurei

3D structure databases

ProteinModelPortaliQ14697.
SMRiQ14697. Positions 239-911.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the glycosyl hydrolase 31 family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiCOG1501.
GeneTreeiENSGT00760000119229.
HOVERGENiHBG051683.
InParanoidiQ14697.
KOiK05546.
OMAiAIDDQLY.
OrthoDBiEOG7VQJDS.
PhylomeDBiQ14697.
TreeFamiTF300337.

Family and domain databases

InterProiIPR011013. Gal_mutarotase_SF_dom.
IPR000322. Glyco_hydro_31.
IPR030458. Glyco_hydro_31_AS.
IPR030459. Glyco_hydro_31_CS.
IPR025887. Glyco_hydro_31_N_dom.
IPR017853. Glycoside_hydrolase_SF.
[Graphical view]
PfamiPF13802. Gal_mutarotas_2. 1 hit.
PF01055. Glyco_hydro_31. 1 hit.
[Graphical view]
SUPFAMiSSF51445. SSF51445. 1 hit.
SSF74650. SSF74650. 2 hits.
PROSITEiPS00129. GLYCOSYL_HYDROL_F31_1. 1 hit.
PS00707. GLYCOSYL_HYDROL_F31_2. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q14697-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAAVAAVAAR RRRSWASLVL AFLGVCLGIT LAVDRSNFKT CEESSFCKRQ
60 70 80 90 100
RSIRPGLSPY RALLDSLQLG PDSLTVHLIH EVTKVLLVLE LQGLQKNMTR
110 120 130 140 150
FRIDELEPRR PRYRVPDVLV ADPPIARLSV SGRDENSVEL TMAEGPYKII
160 170 180 190 200
LTARPFRLDL LEDRSLLLSV NARGLLEFEH QRAPRVSQGS KDPAEGDGAQ
210 220 230 240 250
PEETPRDGDK PEETQGKAEK DEPGAWEETF KTHSDSKPYG PMSVGLDFSL
260 270 280 290 300
PGMEHVYGIP EHADNLRLKV TEGGEPYRLY NLDVFQYELY NPMALYGSVP
310 320 330 340 350
VLLAHNPHRD LGIFWLNAAE TWVDISSNTA GKTLFGKMMD YLQGSGETPQ
360 370 380 390 400
TDVRWMSETG IIDVFLLLGP SISDVFRQYA SLTGTQALPP LFSLGYHQSR
410 420 430 440 450
WNYRDEADVL EVDQGFDDHN LPCDVIWLDI EHADGKRYFT WDPSRFPQPR
460 470 480 490 500
TMLERLASKR RKLVAIVDPH IKVDSGYRVH EELRNLGLYV KTRDGSDYEG
510 520 530 540 550
WCWPGSAGYP DFTNPTMRAW WANMFSYDNY EGSAPNLFVW NDMNEPSVFN
560 570 580 590 600
GPEVTMLKDA QHYGGWEHRD VHNIYGLYVH MATADGLRQR SGGMERPFVL
610 620 630 640 650
ARAFFAGSQR FGAVWTGDNT AEWDHLKISI PMCLSLGLVG LSFCGADVGG
660 670 680 690 700
FFKNPEPELL VRWYQMGAYQ PFFRAHAHLD TGRREPWLLP SQHNDIIRDA
710 720 730 740 750
LGQRYSLLPF WYTLLYQAHR EGIPVMRPLW VQYPQDVTTF NIDDQYLLGD
760 770 780 790 800
ALLVHPVSDS GAHGVQVYLP GQGEVWYDIQ SYQKHHGPQT LYLPVTLSSI
810 820 830 840 850
PVFQRGGTIV PRWMRVRRSS ECMKDDPITL FVALSPQGTA QGELFLDDGH
860 870 880 890 900
TFNYQTRQEF LLRRFSFSGN TLVSSSADPE GHFETPIWIE RVVIIGAGKP
910 920 930 940
AAVVLQTKGS PESRLSFQHD PETSVLVLRK PGINVASDWS IHLR
Length:944
Mass (Da):106,874
Last modified:July 5, 2004 - v3
Checksum:i9E3426FE9A016BF1
GO
Isoform 2 (identifier: Q14697-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     187-187: S → SFSDKVNLTLGSIWDKIKNLFSR

Show »
Length:966
Mass (Da):109,438
Checksum:iD0CD9E47C8E88FB5
GO
Isoform 3 (identifier: Q14697-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     49-52: RQRS → CCWC
     53-944: Missing.

Note: May be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay.
Show »
Length:52
Mass (Da):5,673
Checksum:i012B4BAD808BCD74
GO

Sequence cautioni

The sequence AAH65266.1 differs from that shown. Reason: Erroneous translation. Wrong choice of CDS.Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti400 – 4001R → C in AAH65266 (PubMed:15489334).Curated
Sequence conflicti461 – 4611R → W in AAH65266 (PubMed:15489334).Curated
Sequence conflicti850 – 8501H → Y in CAA04006 (Ref. 1) Curated
Sequence conflicti850 – 8501H → Y in BAA07642 (PubMed:7788527).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti154 – 1541R → W.
Corresponds to variant rs2276296 [ dbSNP | Ensembl ].
VAR_024529
Natural varianti173 – 1731R → Q.
Corresponds to variant rs2276295 [ dbSNP | Ensembl ].
VAR_022086
Natural varianti309 – 3091R → C.
Corresponds to variant rs1063445 [ dbSNP | Ensembl ].
VAR_050272

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei49 – 524RQRS → CCWC in isoform 3. 1 PublicationVSP_039976
Alternative sequencei53 – 944892Missing in isoform 3. 1 PublicationVSP_039977Add
BLAST
Alternative sequencei187 – 1871S → SFSDKVNLTLGSIWDKIKNL FSR in isoform 2. 1 PublicationVSP_010674

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ000332 mRNA. Translation: CAA04006.1.
AF144074 mRNA. Translation: AAF66685.1.
AP001458 Genomic DNA. No translation available.
D42041 mRNA. Translation: BAA07642.1.
BC017433 mRNA. Translation: AAH17433.2.
BC017435 mRNA. Translation: AAH17435.2.
BC065266 mRNA. Translation: AAH65266.1. Sequence problems.
CCDSiCCDS41656.1. [Q14697-2]
CCDS8026.1. [Q14697-1]
RefSeqiNP_001265121.1. NM_001278192.1.
NP_001265122.1. NM_001278193.1.
NP_001265123.1. NM_001278194.1.
NP_938148.1. NM_198334.2. [Q14697-1]
NP_938149.2. NM_198335.3. [Q14697-2]
UniGeneiHs.595071.

Genome annotation databases

EnsembliENST00000346178; ENSP00000340466; ENSG00000089597. [Q14697-2]
ENST00000356638; ENSP00000349053; ENSG00000089597. [Q14697-1]
ENST00000526210; ENSP00000433799; ENSG00000089597. [Q14697-3]
ENST00000532402; ENSP00000432181; ENSG00000089597. [Q14697-3]
ENST00000534613; ENSP00000434921; ENSG00000089597. [Q14697-3]
GeneIDi23193.
KEGGihsa:23193.
UCSCiuc001nua.4. human. [Q14697-2]
uc001nub.4. human. [Q14697-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ000332 mRNA. Translation: CAA04006.1.
AF144074 mRNA. Translation: AAF66685.1.
AP001458 Genomic DNA. No translation available.
D42041 mRNA. Translation: BAA07642.1.
BC017433 mRNA. Translation: AAH17433.2.
BC017435 mRNA. Translation: AAH17435.2.
BC065266 mRNA. Translation: AAH65266.1. Sequence problems.
CCDSiCCDS41656.1. [Q14697-2]
CCDS8026.1. [Q14697-1]
RefSeqiNP_001265121.1. NM_001278192.1.
NP_001265122.1. NM_001278193.1.
NP_001265123.1. NM_001278194.1.
NP_938148.1. NM_198334.2. [Q14697-1]
NP_938149.2. NM_198335.3. [Q14697-2]
UniGeneiHs.595071.

3D structure databases

ProteinModelPortaliQ14697.
SMRiQ14697. Positions 239-911.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi116802. 69 interactions.
IntActiQ14697. 17 interactions.
MINTiMINT-5001279.
STRINGi9606.ENSP00000340466.

Chemistry

BindingDBiQ14697.
ChEMBLiCHEMBL2519.
DrugBankiDB00491. Miglitol.

Protein family/group databases

CAZyiGH31. Glycoside Hydrolase Family 31.

PTM databases

PhosphoSiteiQ14697.

Polymorphism and mutation databases

DMDMi54037162.

2D gel databases

REPRODUCTION-2DPAGEIPI00383581.

Proteomic databases

MaxQBiQ14697.
PaxDbiQ14697.
PRIDEiQ14697.

Protocols and materials databases

DNASUi23193.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000346178; ENSP00000340466; ENSG00000089597. [Q14697-2]
ENST00000356638; ENSP00000349053; ENSG00000089597. [Q14697-1]
ENST00000526210; ENSP00000433799; ENSG00000089597. [Q14697-3]
ENST00000532402; ENSP00000432181; ENSG00000089597. [Q14697-3]
ENST00000534613; ENSP00000434921; ENSG00000089597. [Q14697-3]
GeneIDi23193.
KEGGihsa:23193.
UCSCiuc001nua.4. human. [Q14697-2]
uc001nub.4. human. [Q14697-1]

Organism-specific databases

CTDi23193.
GeneCardsiGC11M062481.
HGNCiHGNC:4138. GANAB.
HPAiHPA026874.
HPA061426.
MIMi104160. gene.
neXtProtiNX_Q14697.
PharmGKBiPA28551.
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG1501.
GeneTreeiENSGT00760000119229.
HOVERGENiHBG051683.
InParanoidiQ14697.
KOiK05546.
OMAiAIDDQLY.
OrthoDBiEOG7VQJDS.
PhylomeDBiQ14697.
TreeFamiTF300337.

Enzyme and pathway databases

UniPathwayiUPA00957.
BRENDAi3.2.1.84. 2681.
ReactomeiREACT_23810. Calnexin/calreticulin cycle.
REACT_23878. N-glycan trimming in the ER and Calnexin/Calreticulin cycle.

Miscellaneous databases

ChiTaRSiGANAB. human.
GeneWikiiGANAB.
GenomeRNAii23193.
NextBioi44685.
PROiQ14697.
SOURCEiSearch...

Gene expression databases

BgeeiQ14697.
CleanExiHS_GANAB.
ExpressionAtlasiQ14697. baseline and differential.
GenevisibleiQ14697. HS.

Family and domain databases

InterProiIPR011013. Gal_mutarotase_SF_dom.
IPR000322. Glyco_hydro_31.
IPR030458. Glyco_hydro_31_AS.
IPR030459. Glyco_hydro_31_CS.
IPR025887. Glyco_hydro_31_N_dom.
IPR017853. Glycoside_hydrolase_SF.
[Graphical view]
PfamiPF13802. Gal_mutarotas_2. 1 hit.
PF01055. Glyco_hydro_31. 1 hit.
[Graphical view]
SUPFAMiSSF51445. SSF51445. 1 hit.
SSF74650. SSF74650. 2 hits.
PROSITEiPS00129. GLYCOSYL_HYDROL_F31_1. 1 hit.
PS00707. GLYCOSYL_HYDROL_F31_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Sequence and analysis of the endoplasmic reticulum protein glucosidase II."
    Stuerzenhofecker B., Nguyenvan P., Soeling H.D.
    Submitted (JUL-1997) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Brain.
  2. "The heterodimeric structure of glucosidase II is required for its activity, solubility, and localization in vivo."
    Pelletier M.F., Marcil A., Sevigny G., Jakob C.A., Tessier D.C., Chevet E., Menard R., Bergeron J.J.M., Thomas D.Y.
    Glycobiology 10:815-827(2000) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 2), FUNCTION, MUTAGENESIS OF ASP-542, INTERACTION WITH PRKCSH, SUBCELLULAR LOCATION, ALTERNATIVE SPLICING.
  3. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  4. "Prediction of the coding sequences of unidentified human genes. III. The coding sequences of 40 new genes (KIAA0081-KIAA0120) deduced by analysis of cDNA clones from human cell line KG-1."
    Nagase T., Miyajima N., Tanaka A., Sazuka T., Seki N., Sato S., Tabata S., Ishikawa K., Kawarabayasi Y., Kotani H., Nomura N.
    DNA Res. 2:37-43(1995) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 2-944 (ISOFORM 1).
    Tissue: Bone marrow.
  5. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 3), NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 457-944 (ISOFORMS 1/2).
    Tissue: Lymph and Uterus.
  6. Lubec G., Afjehi-Sadat L.
    Submitted (MAR-2007) to UniProtKB
    Cited for: PROTEIN SEQUENCE OF 915-929, IDENTIFICATION BY MASS SPECTROMETRY.
    Tissue: Brain and Cajal-Retzius cell.
  7. "Identity of neutral alpha-glucosidase AB and the glycoprotein processing enzyme glucosidase II. Biochemical and genetic studies."
    Martiniuk F., Ellenbogen A., Hirschhorn R.
    J. Biol. Chem. 260:1238-1242(1985) [PubMed] [Europe PMC] [Abstract]
    Cited for: GLYCOSYLATION, TISSUE SPECIFICITY.
  8. "Endoplasmic reticulum glucosidase II is composed of a catalytic subunit, conserved from yeast to mammals, and a tightly bound noncatalytic HDEL-containing subunit."
    Trombetta E.S., Simons J.F., Helenius A.
    J. Biol. Chem. 271:27509-27516(1996) [PubMed] [Europe PMC] [Abstract]
    Cited for: INTERACTION WITH PRKCSH.
  9. Cited for: SUBCELLULAR LOCATION [LARGE SCALE ANALYSIS].
    Tissue: Melanoma.
  10. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  11. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiGANAB_HUMAN
AccessioniPrimary (citable) accession number: Q14697
Secondary accession number(s): A6NC20, Q8WTS9, Q9P0X0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 5, 2004
Last sequence update: July 5, 2004
Last modified: June 24, 2015
This is version 138 of the entry and version 3 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

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Glycosyl hydrolases
    Classification of glycosyl hydrolase families and list of entries
  2. Human chromosome 11
    Human chromosome 11: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  7. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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 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.