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 |