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Protein

Lysosomal alpha-glucosidase

Gene

Gaa

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Essential for the degradation of glygogen to glucose in lysosomes.

Catalytic activityi

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

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei518NucleophilePROSITE-ProRule annotation1
Active sitei521By similarity1

GO - Molecular functioni

GO - Biological processi

  • cardiac muscle contraction Source: MGI
  • diaphragm contraction Source: MGI
  • glycogen catabolic process Source: MGI
  • glycogen metabolic process Source: MGI
  • heart morphogenesis Source: MGI
  • locomotory behavior Source: MGI
  • lysosome organization Source: MGI
  • muscle cell cellular homeostasis Source: MGI
  • neuromuscular process controlling balance Source: MGI
  • neuromuscular process controlling posture Source: MGI
  • regulation of the force of heart contraction Source: MGI
  • striated muscle contraction Source: MGI
  • tissue development Source: MGI
  • vacuolar sequestering Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Glycosidase, Hydrolase

Enzyme and pathway databases

ReactomeiR-MMU-5357572. Lysosomal glycogen catabolism.
R-MMU-6798695. Neutrophil degranulation.

Protein family/group databases

CAZyiGH31. Glycoside Hydrolase Family 31.

Names & Taxonomyi

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

Organism-specific databases

MGIiMGI:95609. Gaa.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: MGI
  • lysosomal membrane Source: MGI
  • lysosome Source: MGI
  • membrane Source: ParkinsonsUK-UCL
Complete GO annotation...

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...)1 Publication1
Glycosylationi233N-linked (GlcNAc...)Sequence analysis1
Glycosylationi390N-linked (GlcNAc...)Sequence analysis1
Glycosylationi470N-linked (GlcNAc...)1 Publication1
Glycosylationi883N-linked (GlcNAc...)Sequence analysis1
Glycosylationi926N-linked (GlcNAc...)Sequence analysis1
Glycosylationi933N-linked (GlcNAc...)Sequence analysis1

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

EPDiP70699.
MaxQBiP70699.
PaxDbiP70699.
PeptideAtlasiP70699.
PRIDEiP70699.

PTM databases

iPTMnetiP70699.
PhosphoSitePlusiP70699.
SwissPalmiP70699.

Expressioni

Gene expression databases

BgeeiENSMUSG00000025579.
CleanExiMM_GAA.
ExpressionAtlasiP70699. baseline and differential.
GenevisibleiP70699. MM.

Interactioni

Protein-protein interaction databases

IntActiP70699. 1 interactor.
MINTiMINT-4100796.
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
Contains 1 P-type (trefoil) domain.PROSITE-ProRule annotation

Keywords - Domaini

Signal

Phylogenomic databases

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

Family and domain databases

CDDicd00111. Trefoil. 1 hit.
Gene3Di4.10.110.10. 1 hit.
InterProiIPR031727. 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.
IPR017853. Glycoside_hydrolase_SF.
IPR017957. P_trefoil_CS.
IPR000519. P_trefoil_dom.
[Graphical view]
PfamiPF13802. Gal_mutarotas_2. 1 hit.
PF01055. Glyco_hydro_31. 1 hit.
PF16863. NtCtMGAM_N. 1 hit.
PF00088. Trefoil. 1 hit.
[Graphical view]
SMARTiSM00018. PD. 1 hit.
[Graphical view]
SUPFAMiSSF51445. SSF51445. 2 hits.
SSF74650. SSF74650. 1 hit.
PROSITEiPS00129. 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.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

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

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.

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

IntActiP70699. 1 interactor.
MINTiMINT-4100796.
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.
OMAiSSEMGYT.
OrthoDBiEOG091G030L.
PhylomeDBiP70699.
TreeFamiTF314577.

Enzyme and pathway databases

ReactomeiR-MMU-5357572. Lysosomal glycogen catabolism.
R-MMU-6798695. Neutrophil degranulation.

Miscellaneous databases

ChiTaRSiGaa. mouse.
PROiP70699.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000025579.
CleanExiMM_GAA.
ExpressionAtlasiP70699. baseline and differential.
GenevisibleiP70699. MM.

Family and domain databases

CDDicd00111. Trefoil. 1 hit.
Gene3Di4.10.110.10. 1 hit.
InterProiIPR031727. 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.
IPR017853. Glycoside_hydrolase_SF.
IPR017957. P_trefoil_CS.
IPR000519. P_trefoil_dom.
[Graphical view]
PfamiPF13802. Gal_mutarotas_2. 1 hit.
PF01055. Glyco_hydro_31. 1 hit.
PF16863. NtCtMGAM_N. 1 hit.
PF00088. Trefoil. 1 hit.
[Graphical view]
SMARTiSM00018. PD. 1 hit.
[Graphical view]
SUPFAMiSSF51445. SSF51445. 2 hits.
SSF74650. SSF74650. 1 hit.
PROSITEiPS00129. 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.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiLYAG_MOUSE
AccessioniPrimary (citable) accession number: P70699
Secondary accession number(s): Q3UJB2, Q8BGI6, Q91Z45
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: August 16, 2004
Last modified: November 30, 2016
This is version 142 of the entry and version 2 of the sequence. [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. Glycosyl hydrolases
    Classification of glycosyl hydrolase families and list of entries
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. 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.