Names & Taxonomy
- Uniprot ID:
- P06280
- Entry Name:
- AGAL_HUMAN
- Status:
- reviewed
- Protein Names:
- Alpha-galactosidase A (EC 3.2.1.22) (Alpha-D-galactosidase A) (Alpha-D-galactoside galactohydrolase) (Melibiase) (Agalsidase)
- Gene Names:
- GLA
- Gene Names Primary:
- GLA
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 429
- Sequence:
- MQLRNPELHLGCALALRFLALVSWDIPGARALDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLL
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Lysosome.
Function
- Catalytic Activity:
- Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids.
- Active Site:
- ACT_SITE 170 170 Nucleophile.
- Gene Ontology Go:
- cytoplasm
extracellular exosome
extracellular region
Golgi apparatus
lysosomal lumen
lysosome
alpha-galactosidase activity
catalytic activity
galactoside binding
hydrolase activity
protein homodimerization activity
raffinose alpha-galactosidase activity
receptor binding
glycosphingolipid catabolic process
glycosphingolipid metabolic process
glycosylceramide catabolic process
negative regulation of nitric oxide biosynthetic process
negative regulation of nitric-oxide synthase activity
oligosaccharide metabolic process
small molecule metabolic process
sphingolipid metabolic process - Gene Ontology Biological Process:
- glycosphingolipid catabolic process
glycosphingolipid metabolic process
glycosylceramide catabolic process
negative regulation of nitric oxide biosynthetic process
negative regulation of nitric-oxide synthase activity
oligosaccharide metabolic process
small molecule metabolic process
sphingolipid metabolic process - Gene Ontology Molecular Function:
- alpha-galactosidase activity
catalytic activity
galactoside binding
hydrolase activity
protein homodimerization activity
raffinose alpha-galactosidase activity
receptor binding - Gene Ontology Cellular Component:
- cytoplasm
extracellular exosome
extracellular region
Golgi apparatus
lysosomal lumen
lysosome - Keywords:
- 3D-structure
Complete proteome
Direct protein sequencing
Disease mutation
Disulfide bond
Glycoprotein
Glycosidase
Hydrolase
Lysosome
Pharmaceutical
RNA editing
Reference proteome
Signal
Publication
- PubMed ID:
- 3036505 2542896 7626884 15772651 15489334 3014515 2892762 2836863 7503918 19159218 21269460 25944712 15003450 7911050 2152885 1846223 2171331 2539398 1315715 7504405 8395937 8069316 7531540 7575533 7759078 7599642 7596372 8738659 8875188 8834244 8931708 8807334 8863162 9105656 9100224 9554750 9452068 9452090 9452111 10208848 10090526 10838196 10666480 11076046 10916280 11295840 11668641 11889412 12694230 12786754 15162124 15712228 16533976 19621417