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Protein

Lysosomal alpha-glucosidase

Gene

Gaa

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans.By similarity

Catalytic activityi

Hydrolysis of terminal, non-reducing (1->4)-linked alpha-D-glucose residues with release of alpha-D-glucose.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei404SubstrateBy similarity1
Active sitei518NucleophilePROSITE-ProRule annotation1
Active sitei521By similarity1
Binding sitei600SubstrateBy similarity1
Binding sitei616SubstrateBy similarity1
Binding sitei674SubstrateBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionGlycosidase, Hydrolase

Enzyme and pathway databases

ReactomeiR-MMU-6798695 Neutrophil degranulation
R-MMU-70221 Glycogen breakdown (glycogenolysis)

Protein family/group databases

CAZyiGH31 Glycoside Hydrolase Family 31

Names & Taxonomyi

Protein namesi
Recommended name:
Lysosomal alpha-glucosidase (EC:3.2.1.20By similarity)
Alternative name(s):
Acid maltase
Gene namesi
Name:Gaa
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 11

Organism-specific databases

MGIiMGI:95609 Gaa

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Lysosome, Membrane

Pathology & Biotechi

Chemistry databases

ChEMBLiCHEMBL1667668

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 27Sequence analysisAdd BLAST27
PropeptideiPRO_000001856928 – 69By similarityAdd BLAST42
ChainiPRO_000001857070 – 953Lysosomal alpha-glucosidaseAdd BLAST884

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi82 ↔ 109PROSITE-ProRule annotation
Disulfide bondi92 ↔ 108PROSITE-ProRule annotation
Disulfide bondi103 ↔ 127PROSITE-ProRule annotation
Glycosylationi140N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi233N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi390N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi470N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi533 ↔ 558By similarity
Disulfide bondi647 ↔ 658By similarity
Glycosylationi883N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi926N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi933N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

EPDiP70699
MaxQBiP70699
PaxDbiP70699
PeptideAtlasiP70699
PRIDEiP70699

PTM databases

iPTMnetiP70699
PhosphoSitePlusiP70699
SwissPalmiP70699

Expressioni

Gene expression databases

BgeeiENSMUSG00000025579 Expressed in 331 organ(s), highest expression level in arcuate nucleus of hypothalamus
CleanExiMM_GAA
ExpressionAtlasiP70699 baseline and differential
GenevisibleiP70699 MM

Interactioni

Protein-protein interaction databases

BioGridi199792, 1 interactor
IntActiP70699, 1 interactor
MINTiP70699
STRINGi10090.ENSMUSP00000026666

Chemistry databases

BindingDBiP70699

Structurei

3D structure databases

ProteinModelPortaliP70699
SMRiP70699
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini80 – 131P-typePROSITE-ProRule annotationAdd BLAST52

Sequence similaritiesi

Belongs to the glycosyl hydrolase 31 family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG1065 Eukaryota
COG1501 LUCA
GeneTreeiENSGT00760000119229
HOGENOMiHOG000041175
HOVERGENiHBG006297
InParanoidiP70699
KOiK12316
OMAiTINVHLR
OrthoDBiEOG091G030L
PhylomeDBiP70699
TreeFamiTF314577

Family and domain databases

CDDicd00111 Trefoil, 1 hit
Gene3Di2.60.40.1180, 2 hits
InterProiView protein in InterPro
IPR031727 Gal_mutarotase_N
IPR011013 Gal_mutarotase_sf_dom
IPR000322 Glyco_hydro_31
IPR030458 Glyco_hydro_31_AS
IPR030459 Glyco_hydro_31_CS
IPR025887 Glyco_hydro_31_N_dom
IPR013780 Glyco_hydro_b
IPR017853 Glycoside_hydrolase_SF
IPR017957 P_trefoil_CS
IPR000519 P_trefoil_dom
PfamiView protein in Pfam
PF13802 Gal_mutarotas_2, 1 hit
PF01055 Glyco_hydro_31, 1 hit
PF16863 NtCtMGAM_N, 1 hit
PF00088 Trefoil, 1 hit
SMARTiView protein in SMART
SM00018 PD, 1 hit
SUPFAMiSSF51445 SSF51445, 1 hit
SSF74650 SSF74650, 1 hit
PROSITEiView protein in PROSITE
PS00129 GLYCOSYL_HYDROL_F31_1, 1 hit
PS00707 GLYCOSYL_HYDROL_F31_2, 1 hit
PS00025 P_TREFOIL_1, 1 hit
PS51448 P_TREFOIL_2, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 4 potential isoforms that are computationally mapped.Show allAlign All

P70699-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MNIRKPLCSN SVVGACTLIS LTTAVILGHL MLRELMLLPQ DLHESSSGLW
60 70 80 90 100
KTYRPHHQEG YKPGPLHIQE QTEQPKEAPT QCDVPPSSRF DCAPDKGISQ
110 120 130 140 150
EQCEARGCCY VPAGQVLKEP QIGQPWCFFP PSYPSYRLEN LSSTESGYTA
160 170 180 190 200
TLTRTSPTFF PKDVLTLQLE VLMETDSRLH FKIKDPASKR YEVPLETPRV
210 220 230 240 250
LSQAPSPLYS VEFSEEPFGV IVRRKLGGRV LLNTTVAPLF FADQFLQLST
260 270 280 290 300
SLPSQHITGL GEHLSPLMLS TDWARITLWN RDTPPSQGTN LYGSHPFYLA
310 320 330 340 350
LEDGGLAHGV FLLNSNAMDV ILQPSPALTW RSTGGILDVY VFLGPEPKSV
360 370 380 390 400
VQQYLDVVGY PFMPPYWGLG FHLCRWGYSS TAIVRQVVEN MTRTHFPLDV
410 420 430 440 450
QWNDLDYMDA RRDFTFNQDS FADFPDMVRE LHQDGRRYMM IVDPAISSAG
460 470 480 490 500
PAGSYRPYDE GLRRGVFITN ETGQPLIGKV WPGTTAFPDF TNPETLDWWQ
510 520 530 540 550
DMVSEFHAQV PFDGMWLDMN EPSNFVRGSQ QGCPNNELEN PPYVPGVVGG
560 570 580 590 600
ILQAATICAS SHQFLSTHYN LHNLYGLTEA IASSRALVKT RGTRPFVISR
610 620 630 640 650
STFSGHGRYA GHWTGDVRSS WEHLAYSVPD ILQFNLLGVP LVGADICGFI
660 670 680 690 700
GDTSEELCVR WTQLGAFYPF MRNHNDLNSV PQEPYRFSET AQQAMRKAFA
710 720 730 740 750
LRYALLPYLY TLFHRAHVRG DTVARPLFLE FPEDPSTWSV DRQLLWGPAL
760 770 780 790 800
LITPVLEPGK TEVTGYFPKG TWYNMQMVSV DSLGTLPSPS SASSFRSAVQ
810 820 830 840 850
SKGQWLTLEA PLDTINVHLR EGYIIPLQGP SLTTTESRKQ PMALAVALTA
860 870 880 890 900
SGEADGELFW DDGESLAVLE RGAYTLVTFS AKNNTIVNKL VRVTKEGAEL
910 920 930 940 950
QLREVTVLGV ATAPTQVLSN GIPVSNFTYS PDNKSLAIPV SLLMGELFQI

SWS
Length:953
Mass (Da):106,248
Last modified:August 16, 2004 - v2
Checksum:i956B89685FB5FF81
GO

Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A2AFL3A2AFL3_MOUSE
Lysosomal alpha-glucosidase
Gaa
231Annotation score:
A2AFL5A2AFL5_MOUSE
Lysosomal alpha-glucosidase
Gaa
272Annotation score:
F6R5R5F6R5R5_MOUSE
Lysosomal alpha-glucosidase
Gaa
207Annotation score:
F6VEG4F6VEG4_MOUSE
Lysosomal alpha-glucosidase
Gaa
106Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti62K → E in AAB06943 (Ref. 1) Curated1
Sequence conflicti254S → A in AAB06943 (Ref. 1) Curated1
Sequence conflicti430 – 431EL → DV in AAB06943 (Ref. 1) Curated2
Sequence conflicti434D → G (Ref. 1) Curated1
Sequence conflicti434D → G (PubMed:15489334).Curated1
Sequence conflicti481W → C in AAB06943 (Ref. 1) Curated1
Sequence conflicti615G → E in AAB06943 (Ref. 1) Curated1
Sequence conflicti619S → T in AAB06943 (Ref. 1) Curated1
Sequence conflicti732P → R in AAB06943 (Ref. 1) Curated1
Sequence conflicti777M → V (Ref. 1) Curated1
Sequence conflicti777M → V (PubMed:15489334).Curated1
Sequence conflicti871R → H in AAB06943 (Ref. 1) Curated1
Sequence conflicti903R → K in AAB06943 (Ref. 1) Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U49351 mRNA Translation: AAB06943.1
AK052211 mRNA Translation: BAC34888.1
AK088481 mRNA Translation: BAC40382.1
AK139333 mRNA Translation: BAE23960.1
AK146538 mRNA Translation: BAE27243.1
AK150970 mRNA Translation: BAE30001.1
BC010210 mRNA Translation: AAH10210.1
CCDSiCCDS25713.1
RefSeqiNP_001152796.1, NM_001159324.2
NP_032090.3, NM_008064.4
UniGeneiMm.4793

Genome annotation databases

EnsembliENSMUST00000026666; ENSMUSP00000026666; ENSMUSG00000025579
ENSMUST00000106259; ENSMUSP00000101866; ENSMUSG00000025579
GeneIDi14387
KEGGimmu:14387
UCSCiuc007mqg.2 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U49351 mRNA Translation: AAB06943.1
AK052211 mRNA Translation: BAC34888.1
AK088481 mRNA Translation: BAC40382.1
AK139333 mRNA Translation: BAE23960.1
AK146538 mRNA Translation: BAE27243.1
AK150970 mRNA Translation: BAE30001.1
BC010210 mRNA Translation: AAH10210.1
CCDSiCCDS25713.1
RefSeqiNP_001152796.1, NM_001159324.2
NP_032090.3, NM_008064.4
UniGeneiMm.4793

3D structure databases

ProteinModelPortaliP70699
SMRiP70699
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi199792, 1 interactor
IntActiP70699, 1 interactor
MINTiP70699
STRINGi10090.ENSMUSP00000026666

Chemistry databases

BindingDBiP70699
ChEMBLiCHEMBL1667668

Protein family/group databases

CAZyiGH31 Glycoside Hydrolase Family 31

PTM databases

iPTMnetiP70699
PhosphoSitePlusiP70699
SwissPalmiP70699

Proteomic databases

EPDiP70699
MaxQBiP70699
PaxDbiP70699
PeptideAtlasiP70699
PRIDEiP70699

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000026666; ENSMUSP00000026666; ENSMUSG00000025579
ENSMUST00000106259; ENSMUSP00000101866; ENSMUSG00000025579
GeneIDi14387
KEGGimmu:14387
UCSCiuc007mqg.2 mouse

Organism-specific databases

CTDi2548
MGIiMGI:95609 Gaa

Phylogenomic databases

eggNOGiKOG1065 Eukaryota
COG1501 LUCA
GeneTreeiENSGT00760000119229
HOGENOMiHOG000041175
HOVERGENiHBG006297
InParanoidiP70699
KOiK12316
OMAiTINVHLR
OrthoDBiEOG091G030L
PhylomeDBiP70699
TreeFamiTF314577

Enzyme and pathway databases

ReactomeiR-MMU-6798695 Neutrophil degranulation
R-MMU-70221 Glycogen breakdown (glycogenolysis)

Miscellaneous databases

ChiTaRSiGaa mouse
PROiPR:P70699
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000025579 Expressed in 331 organ(s), highest expression level in arcuate nucleus of hypothalamus
CleanExiMM_GAA
ExpressionAtlasiP70699 baseline and differential
GenevisibleiP70699 MM

Family and domain databases

CDDicd00111 Trefoil, 1 hit
Gene3Di2.60.40.1180, 2 hits
InterProiView protein in InterPro
IPR031727 Gal_mutarotase_N
IPR011013 Gal_mutarotase_sf_dom
IPR000322 Glyco_hydro_31
IPR030458 Glyco_hydro_31_AS
IPR030459 Glyco_hydro_31_CS
IPR025887 Glyco_hydro_31_N_dom
IPR013780 Glyco_hydro_b
IPR017853 Glycoside_hydrolase_SF
IPR017957 P_trefoil_CS
IPR000519 P_trefoil_dom
PfamiView protein in Pfam
PF13802 Gal_mutarotas_2, 1 hit
PF01055 Glyco_hydro_31, 1 hit
PF16863 NtCtMGAM_N, 1 hit
PF00088 Trefoil, 1 hit
SMARTiView protein in SMART
SM00018 PD, 1 hit
SUPFAMiSSF51445 SSF51445, 1 hit
SSF74650 SSF74650, 1 hit
PROSITEiView protein in PROSITE
PS00129 GLYCOSYL_HYDROL_F31_1, 1 hit
PS00707 GLYCOSYL_HYDROL_F31_2, 1 hit
PS00025 P_TREFOIL_1, 1 hit
PS51448 P_TREFOIL_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiLYAG_MOUSE
AccessioniPrimary (citable) accession number: P70699
Secondary accession number(s): Q3UJB2, Q8BGI6, Q91Z45
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: August 16, 2004
Last modified: November 7, 2018
This is version 155 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. Glycosyl hydrolases
    Classification of glycosyl hydrolase families and list of entries
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