CPLM 1.0 - Compendium of Protein Lysine Modification
TagContent
CPLM ID CPLM-003780
UniProt Accession
Genbank Protein ID
Genbank Nucleotide ID
Protein Name
 Lysosomal alpha-glucosidase 
Protein Synonyms/Alias
 Acid maltase; Aglucosidase alfa; 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase 
Gene Name
 GAA 
Gene Synonyms/Alias
  
Created Date
 July 27, 2013 
Organism
 Homo sapiens (Human) 
NCBI Taxa ID
 9606 
Lysine Modification
Position
Peptide
Type
References
162TTPTFFPKDILTLRLubiquitination[1]
Reference
 [1] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961] 
Functional Description
 Essential for the degradation of glygogen to glucose in lysosomes. 
Sequence Annotation
 DOMAIN 80 131 P-type.
 ACT_SITE 518 518 Nucleophile.
 ACT_SITE 521 521 By similarity.
 CARBOHYD 140 140 N-linked (GlcNAc...).
 CARBOHYD 233 233 N-linked (GlcNAc...).
 CARBOHYD 390 390 N-linked (GlcNAc...).
 CARBOHYD 470 470 N-linked (GlcNAc...).
 CARBOHYD 652 652 N-linked (GlcNAc...).
 CARBOHYD 882 882 N-linked (GlcNAc...).
 CARBOHYD 925 925 N-linked (GlcNAc...).
 DISULFID 82 109 By similarity.
 DISULFID 92 108 By similarity.
 DISULFID 103 127 By similarity.  
Keyword
 Complete proteome; Direct protein sequencing; Disease mutation; Disulfide bond; Glycogen storage disease; Glycoprotein; Glycosidase; Hydrolase; Lysosome; Membrane; Phosphoprotein; Polymorphism; Reference proteome; Signal. 
Sequence Source
 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 
Protein Length
 952 AA 
Protein Sequence
MGVRHPPCSH RLLAVCALVS LATAALLGHI LLHDFLLVPR ELSGSSPVLE ETHPAHQQGA 60
SRPGPRDAQA HPGRPRAVPT QCDVPPNSRF DCAPDKAITQ EQCEARGCCY IPAKQGLQGA 120
QMGQPWCFFP PSYPSYKLEN LSSSEMGYTA TLTRTTPTFF PKDILTLRLD VMMETENRLH 180
FTIKDPANRR YEVPLETPHV HSRAPSPLYS VEFSEEPFGV IVRRQLDGRV LLNTTVAPLF 240
FADQFLQLST SLPSQYITGL AEHLSPLMLS TSWTRITLWN RDLAPTPGAN LYGSHPFYLA 300
LEDGGSAHGV FLLNSNAMDV VLQPSPALSW RSTGGILDVY IFLGPEPKSV VQQYLDVVGY 360
PFMPPYWGLG FHLCRWGYSS TAITRQVVEN MTRAHFPLDV QWNDLDYMDS RRDFTFNKDG 420
FRDFPAMVQE LHQGGRRYMM IVDPAISSSG PAGSYRPYDE GLRRGVFITN ETGQPLIGKV 480
WPGSTAFPDF TNPTALAWWE DMVAEFHDQV PFDGMWIDMN EPSNFIRGSE DGCPNNELEN 540
PPYVPGVVGG TLQAATICAS SHQFLSTHYN LHNLYGLTEA IASHRALVKA RGTRPFVISR 600
STFAGHGRYA GHWTGDVWSS WEQLASSVPE ILQFNLLGVP LVGADVCGFL GNTSEELCVR 660
WTQLGAFYPF MRNHNSLLSL PQEPYSFSEP AQQAMRKALT LRYALLPHLY TLFHQAHVAG 720
ETVARPLFLE FPKDSSTWTV DHQLLWGEAL LITPVLQAGK AEVTGYFPLG TWYDLQTVPV 780
EALGSLPPPP AAPREPAIHS EGQWVTLPAP LDTINVHLRA GYIIPLQGPG LTTTESRQQP 840
MALAVALTKG GEARGELFWD DGESLEVLER GAYTQVIFLA RNNTIVNELV RVTSEGAGLQ 900
LQKVTVLGVA TAPQQVLSNG VPVSNFTYSP DTKVLDICVS LLMGEQFLVS WC 952 
Gene Ontology
 GO:0005765; C:lysosomal membrane; IEA:UniProtKB-SubCell.
 GO:0005764; C:lysosome; IDA:BHF-UCL.
 GO:0004558; F:alpha-glucosidase activity; IDA:BHF-UCL.
 GO:0030246; F:carbohydrate binding; IEA:InterPro.
 GO:0032450; F:maltose alpha-glucosidase activity; IEA:EC.
 GO:0060048; P:cardiac muscle contraction; IMP:BHF-UCL.
 GO:0002086; P:diaphragm contraction; IMP:BHF-UCL.
 GO:0006006; P:glucose metabolic process; IC:BHF-UCL.
 GO:0005980; P:glycogen catabolic process; IDA:BHF-UCL.
 GO:0007626; P:locomotory behavior; IEA:Compara.
 GO:0007040; P:lysosome organization; IMP:BHF-UCL.
 GO:0000023; P:maltose metabolic process; IC:BHF-UCL.
 GO:0046716; P:muscle cell homeostasis; IEA:Compara.
 GO:0050885; P:neuromuscular process controlling balance; IEA:Compara.
 GO:0050884; P:neuromuscular process controlling posture; IEA:Compara.
 GO:0002026; P:regulation of the force of heart contraction; IEA:Compara.
 GO:0005985; P:sucrose metabolic process; IC:BHF-UCL.
 GO:0043587; P:tongue morphogenesis; IMP:BHF-UCL.
 GO:0043181; P:vacuolar sequestering; IMP:BHF-UCL.
 GO:0055010; P:ventricular cardiac muscle tissue morphogenesis; IMP:BHF-UCL. 
Interpro
 IPR011013; Gal_mutarotase_SF_dom.
 IPR000322; Glyco_hydro_31.
 IPR025887; Glyco_hydro_31_N_dom.
 IPR017853; Glycoside_hydrolase_SF.
 IPR000519; P_trefoil.
 IPR017957; P_trefoil_CS. 
Pfam
 PF13802; Gal_mutarotas_2
 PF01055; Glyco_hydro_31
 PF00088; Trefoil 
SMART
 SM00018; PD 
PROSITE
 PS00129; GLYCOSYL_HYDROL_F31_1
 PS00707; GLYCOSYL_HYDROL_F31_2
 PS00025; P_TREFOIL_1
 PS51448; P_TREFOIL_2 
PRINTS