| 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
|
|
|
Transmembrane Regions
|
|
| Protein Sequence
|
>Lysosomal alpha-glucosidase
MMRWPPCSRPLLGVCTLLSLALLGHILLHDLEVVPRELRGFSQDEIHQACQPGASSPECR
GSPRAAPTQCDLPPNSRFDCAPDKGITPQQCEARGCCYMPAEWPPDAQMGQPWCFFPPSY
PSYRLENLTTTETGYTATLTRAVPTFFPKDIMTLRLDMLMETESRLHFTIKDPANRRYEV
PLETPRVYSQAPFTLYSVEFSEEPFGVVVRRKLDGRVLLNTTVAPLFFADQFLQLSTSLP
SQHITGLAEHLGSLMLSTNWTKITLWNRDIAPEPNVNLYGSHPFYLVLEDGGLAHGVFLL
NSNAMDVVLQPSPALSWRSTGGILDVYIFLGPEPKSVVQQYLDVVGYPFMPPYWGLGFHL
CRWGYSTSAITRQVVENMTRAYFPLDVQWNDLDYMDARRDFTFNKDHFGDFPAMVQELHQ
GGRRYIMIVDPAISSSGPAGTYRPYDEGLRRGVFITNETGQPLIGQVWPGLTAFPDFTNP
ETLDWWQDMVTEFHAQVPFDGMWIDMNEPSNFVRGSVDGCPDNSLENPPYLPGVVGGTLR
AATICASSHQFLSTHYDLHNLYGLTEALASHRALVKARGMRPFVISRSTFAGHGRYSGHW
TGDVWSNWEQLSYSVPEILLFNLLGVPLVGADICGFLGNTSEELCVRWTQLGAFYPFMRN
HNALNSQPQEPYRFSETAQQAMRKAFTLRYVLLPYLYTLFHRAHVRGETVARPLFLEFPE
DPSTWTVDRQLLWGEALLITPVLEAEKVEVTGYFPQGTWYDLQTVPMEAFGSLPPPAPLT
SVIHSKGQWVTLSAPLDTINVHLRAGHIIPMQGPALTTTESRKQHMALAVALTASGEAQG
ELFWDDGESLGVLDGGDYTQLIFLAKNNTFVNKLVHVSSEGASLQLRNVTVLGVATAPQQ
VLCNSVPVSNFTFSPDTETLAIPVSLTMGEQFVISWS
|
| External Links |
| 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 |