LMPD Database

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LMP000589

UniProt Annotations

Entry Information
Gene Namegalactosidase, alpha
Protein EntryAGAL_HUMAN
UniProt IDP06280
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityHydrolysis of terminal, non-reducing alpha-D- galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids.
DiseaseFabry disease (FD) [MIM
PharmaceuticalAvailable under the names Replagal (from Shire) and Fabrazyme (from Genzyme). Used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. The differences between Replagal (also known as agalsidase alpha) and Fabrazyme (also known as agalsidase beta) lies in the glycosylation patterns. Agalsidase beta is produced in the hamster CHO cell line while agalsidase alpha is produced in human cell lines.
Rna EditingModified_positions=396 ; Note=Partially edited.;
SimilarityBelongs to the glycosyl hydrolase 27 family.
Subcellular LocationLysosome.
SubunitHomodimer.