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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)LengthDescriptionGraphical viewFeature identifierActions
Active sitei518 – 5181NucleophilePROSITE-ProRule annotation
Active sitei521 – 5211By similarity

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

ReactomeiREACT_360650. Lysosomal glycogen catabolism.

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
ProteomesiUP000000589 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

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 2727Sequence AnalysisAdd
BLAST
Propeptidei28 – 6942By similarityPRO_0000018569Add
BLAST
Chaini70 – 953884Lysosomal alpha-glucosidasePRO_0000018570Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi82 ↔ 109PROSITE-ProRule annotation
Disulfide bondi92 ↔ 108PROSITE-ProRule annotation
Disulfide bondi103 ↔ 127PROSITE-ProRule annotation
Glycosylationi140 – 1401N-linked (GlcNAc...)1 Publication
Glycosylationi233 – 2331N-linked (GlcNAc...)Sequence Analysis
Glycosylationi390 – 3901N-linked (GlcNAc...)Sequence Analysis
Glycosylationi470 – 4701N-linked (GlcNAc...)1 Publication
Glycosylationi883 – 8831N-linked (GlcNAc...)Sequence Analysis
Glycosylationi926 – 9261N-linked (GlcNAc...)Sequence Analysis
Glycosylationi933 – 9331N-linked (GlcNAc...)Sequence Analysis

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

MaxQBiP70699.
PaxDbiP70699.
PRIDEiP70699.

PTM databases

PhosphoSiteiP70699.

Expressioni

Gene expression databases

BgeeiP70699.
CleanExiMM_GAA.
ExpressionAtlasiP70699. baseline and differential.
GenevestigatoriP70699.

Interactioni

Protein-protein interaction databases

IntActiP70699. 1 interaction.
MINTiMINT-4100796.

Structurei

3D structure databases

ProteinModelPortaliP70699.
SMRiP70699. Positions 89-953.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini80 – 13152P-typePROSITE-ProRule annotationAdd
BLAST

Sequence similaritiesi

Belongs to the glycosyl hydrolase 31 family.Curated
Contains 1 P-type (trefoil) domain.PROSITE-ProRule annotation

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiCOG1501.
GeneTreeiENSGT00760000119229.
HOGENOMiHOG000041175.
HOVERGENiHBG006297.
InParanoidiP70699.
KOiK12316.
OMAiEPSNFVE.
OrthoDBiEOG77HDD0.
PhylomeDBiP70699.
TreeFamiTF314577.

Family and domain databases

Gene3Di4.10.110.10. 1 hit.
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.
IPR000519. P_trefoil.
IPR017957. P_trefoil_CS.
[Graphical view]
PfamiPF13802. Gal_mutarotas_2. 1 hit.
PF01055. Glyco_hydro_31. 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)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti62 – 621K → E in AAB06943 (Ref. 1) Curated
Sequence conflicti254 – 2541S → A in AAB06943 (Ref. 1) Curated
Sequence conflicti430 – 4312EL → DV in AAB06943 (Ref. 1) Curated
Sequence conflicti434 – 4341D → G (Ref. 1) Curated
Sequence conflicti434 – 4341D → G (PubMed:15489334).Curated
Sequence conflicti481 – 4811W → C in AAB06943 (Ref. 1) Curated
Sequence conflicti615 – 6151G → E in AAB06943 (Ref. 1) Curated
Sequence conflicti619 – 6191S → T in AAB06943 (Ref. 1) Curated
Sequence conflicti732 – 7321P → R in AAB06943 (Ref. 1) Curated
Sequence conflicti777 – 7771M → V (Ref. 1) Curated
Sequence conflicti777 – 7771M → V (PubMed:15489334).Curated
Sequence conflicti871 – 8711R → H in AAB06943 (Ref. 1) Curated
Sequence conflicti903 – 9031R → K in AAB06943 (Ref. 1) Curated

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_001159324.1.
NP_032090.3. NM_008064.3.
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_001159324.1.
NP_032090.3. NM_008064.3.
UniGeneiMm.4793.

3D structure databases

ProteinModelPortaliP70699.
SMRiP70699. Positions 89-953.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP70699. 1 interaction.
MINTiMINT-4100796.

Chemistry

BindingDBiP70699.
ChEMBLiCHEMBL1667668.

Protein family/group databases

CAZyiGH31. Glycoside Hydrolase Family 31.

PTM databases

PhosphoSiteiP70699.

Proteomic databases

MaxQBiP70699.
PaxDbiP70699.
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

eggNOGiCOG1501.
GeneTreeiENSGT00760000119229.
HOGENOMiHOG000041175.
HOVERGENiHBG006297.
InParanoidiP70699.
KOiK12316.
OMAiEPSNFVE.
OrthoDBiEOG77HDD0.
PhylomeDBiP70699.
TreeFamiTF314577.

Enzyme and pathway databases

ReactomeiREACT_360650. Lysosomal glycogen catabolism.

Miscellaneous databases

ChiTaRSiGaa. mouse.
NextBioi285901.
PROiP70699.
SOURCEiSearch...

Gene expression databases

BgeeiP70699.
CleanExiMM_GAA.
ExpressionAtlasiP70699. baseline and differential.
GenevestigatoriP70699.

Family and domain databases

Gene3Di4.10.110.10. 1 hit.
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.
IPR000519. P_trefoil.
IPR017957. P_trefoil_CS.
[Graphical view]
PfamiPF13802. Gal_mutarotas_2. 1 hit.
PF01055. Glyco_hydro_31. 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...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning and characterization of the mouse liver cDNA encoding lysosomal alpha-glucosidase."
    Ding J.H., Yang B.Z., Reuser A.J.J., Roe C.R.
    Submitted (SEP-1996) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: BALB/c.
    Tissue: Liver.
  2. "The transcriptional landscape of the mammalian genome."
    Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
    , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
    Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: C57BL/6J and NOD.
    Tissue: Bone marrow, Brain cortex, Heart and Thymus.
  3. "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].
    Strain: FVB/N.
    Tissue: Mammary tumor.
  4. Lubec G., Sunyer B., Chen W.-Q.
    Submitted (JAN-2009) to UniProtKB
    Cited for: PROTEIN SEQUENCE OF 376-385, IDENTIFICATION BY MASS SPECTROMETRY.
    Strain: OF1.
    Tissue: Hippocampus.
  5. "Mass-spectrometric identification and relative quantification of N-linked cell surface glycoproteins."
    Wollscheid B., Bausch-Fluck D., Henderson C., O'Brien R., Bibel M., Schiess R., Aebersold R., Watts J.D.
    Nat. Biotechnol. 27:378-386(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-140 AND ASN-470.

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: May 27, 2015
This is version 130 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

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.