Showing Protein Lysosomal alpha-glucosidase (BMDBP00803)

IdentificationBiological propertiesGene propertiesProtein propertiesExternal linksReferencesXMLShow 8 metabolites
Identification
BMDB Protein ID BMDBP00803
Secondary Accession Numbers None
Name Lysosomal alpha-glucosidase
Synonyms Not Available
Gene Name GAA
Protein Type Enzyme
Biological Properties
General Function Carbohydrate transport and metabolism
Specific Function Essential for the degradation of glycogen in lysosomes (PubMed:10723725). Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans.
Pathways
  • Galactose Metabolism
  • Starch and Sucrose Metabolism
Reactions
Sucrose + Water → D-Galactose + D-Fructose details
Alpha-D-Glucose + D-Fructose → Sucrose + Water details
4 Alpha-D-Glucose → Glycogen details
Dextrin →3 Alpha-D-Glucose details
D-Maltose →2 Alpha-D-Glucose details
GO Classification Not Available
Cellular Location Not Available
Gene Properties
Chromosome Location 19
Locus Not Available
SNPs GAA
Gene Sequence Not Available
Protein Properties
Number of Residues 937
Molecular Weight 104757.0
Theoretical pI 5.23
Pfam Domain Function Not Available
Signals
  • 1-23
Transmembrane Regions
  • 418-438
Protein Sequence 
>Lysosomal alpha-glucosidase
MMRWPPCSRPLLGVCTLLSLALLGHILLHDLEVVPRELRGFSQDEIHQACQPGASSPECR
GSPRAAPTQCDLPPNSRFDCAPDKGITPQQCEARGCCYMPAEWPPDAQMGQPWCFFPPSY
PSYRLENLTTTETGYTATLTRAVPTFFPKDIMTLRLDMLMETESRLHFTIKDPANRRYEV
PLETPRVYSQAPFTLYSVEFSEEPFGVVVRRKLDGRVLLNTTVAPLFFADQFLQLSTSLP
SQHITGLAEHLGSLMLSTNWTKITLWNRDIAPEPNVNLYGSHPFYLVLEDGGLAHGVFLL
NSNAMDVVLQPSPALSWRSTGGILDVYIFLGPEPKSVVQQYLDVVGYPFMPPYWGLGFHL
CRWGYSTSAITRQVVENMTRAYFPLDVQWNDLDYMDARRDFTFNKDHFGDFPAMVQELHQ
GGRRYIMIVDPAISSSGPAGTYRPYDEGLRRGVFITNETGQPLIGQVWPGLTAFPDFTNP
ETLDWWQDMVTEFHAQVPFDGMWIDMNEPSNFVRGSVDGCPDNSLENPPYLPGVVGGTLR
AATICASSHQFLSTHYDLHNLYGLTEALASHRALVKARGMRPFVISRSTFAGHGRYSGHW
TGDVWSNWEQLSYSVPEILLFNLLGVPLVGADICGFLGNTSEELCVRWTQLGAFYPFMRN
HNALNSQPQEPYRFSETAQQAMRKAFTLRYVLLPYLYTLFHRAHVRGETVARPLFLEFPE
DPSTWTVDRQLLWGEALLITPVLEAEKVEVTGYFPQGTWYDLQTVPMEAFGSLPPPAPLT
SVIHSKGQWVTLSAPLDTINVHLRAGHIIPMQGPALTTTESRKQHMALAVALTASGEAQG
ELFWDDGESLGVLDGGDYTQLIFLAKNNTFVNKLVHVSSEGASLQLRNVTVLGVATAPQQ
VLCNSVPVSNFTFSPDTETLAIPVSLTMGEQFVISWS
GenBank ID Protein AAF81636.1
UniProtKB/Swiss-Prot ID Q9MYM4
UniProtKB/Swiss-Prot Entry Name LYAG_BOVIN
PDB IDs Not Available
GenBank Gene ID AF171665
GeneCard ID GAA
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available