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Human lysosomal beta-hexosaminidase isoform B in complex with (2R,3R,4S,5R)-2-Acetamido-3,4-Dihydroxy-5-Hydroxymethyl-Piperidinium Chloride (GalNAc-isofagomine)

Functional Information from GO Data
ChainGOidnamespacecontents
A0001501biological_processskeletal system development
A0001669cellular_componentacrosomal vesicle
A0004553molecular_functionhydrolase activity, hydrolyzing O-glycosyl compounds
A0004563molecular_functionbeta-N-acetylhexosaminidase activity
A0005515molecular_functionprotein binding
A0005576cellular_componentextracellular region
A0005615cellular_componentextracellular space
A0005764cellular_componentlysosome
A0005975biological_processcarbohydrate metabolic process
A0006629biological_processlipid metabolic process
A0006687biological_processglycosphingolipid metabolic process
A0006689biological_processganglioside catabolic process
A0006874biological_processintracellular calcium ion homeostasis
A0007040biological_processlysosome organization
A0007338biological_processsingle fertilization
A0007341biological_processpenetration of zona pellucida
A0007605biological_processsensory perception of sound
A0007626biological_processlocomotory behavior
A0008049biological_processmale courtship behavior
A0008360biological_processregulation of cell shape
A0008375molecular_functionacetylglucosaminyltransferase activity
A0008654biological_processphospholipid biosynthetic process
A0009313biological_processoligosaccharide catabolic process
A0015929molecular_functionhexosaminidase activity
A0016020cellular_componentmembrane
A0016787molecular_functionhydrolase activity
A0016798molecular_functionhydrolase activity, acting on glycosyl bonds
A0019538biological_processprotein metabolic process
A0019915biological_processlipid storage
A0019953biological_processsexual reproduction
A0030203biological_processglycosaminoglycan metabolic process
A0030207biological_processchondroitin sulfate catabolic process
A0030209biological_processdermatan sulfate catabolic process
A0030214biological_processhyaluronan catabolic process
A0031410cellular_componentcytoplasmic vesicle
A0035578cellular_componentazurophil granule lumen
A0042552biological_processmyelination
A0042582cellular_componentazurophil granule
A0042802molecular_functionidentical protein binding
A0043202cellular_componentlysosomal lumen
A0043615biological_processastrocyte cell migration
A0045944biological_processpositive regulation of transcription by RNA polymerase II
A0048477biological_processoogenesis
A0050885biological_processneuromuscular process controlling balance
A0050905biological_processneuromuscular process
A0051651biological_processmaintenance of location in cell
A0060473cellular_componentcortical granule
A0070050biological_processneuron cellular homeostasis
A0070062cellular_componentextracellular exosome
A0102148molecular_functionN-acetyl-beta-D-galactosaminidase activity
A1905379cellular_componentbeta-N-acetylhexosaminidase complex
B0001501biological_processskeletal system development
B0001669cellular_componentacrosomal vesicle
B0004553molecular_functionhydrolase activity, hydrolyzing O-glycosyl compounds
B0004563molecular_functionbeta-N-acetylhexosaminidase activity
B0005515molecular_functionprotein binding
B0005576cellular_componentextracellular region
B0005615cellular_componentextracellular space
B0005764cellular_componentlysosome
B0005975biological_processcarbohydrate metabolic process
B0006629biological_processlipid metabolic process
B0006687biological_processglycosphingolipid metabolic process
B0006689biological_processganglioside catabolic process
B0006874biological_processintracellular calcium ion homeostasis
B0007040biological_processlysosome organization
B0007338biological_processsingle fertilization
B0007341biological_processpenetration of zona pellucida
B0007605biological_processsensory perception of sound
B0007626biological_processlocomotory behavior
B0008049biological_processmale courtship behavior
B0008360biological_processregulation of cell shape
B0008375molecular_functionacetylglucosaminyltransferase activity
B0008654biological_processphospholipid biosynthetic process
B0009313biological_processoligosaccharide catabolic process
B0015929molecular_functionhexosaminidase activity
B0016020cellular_componentmembrane
B0016787molecular_functionhydrolase activity
B0016798molecular_functionhydrolase activity, acting on glycosyl bonds
B0019538biological_processprotein metabolic process
B0019915biological_processlipid storage
B0019953biological_processsexual reproduction
B0030203biological_processglycosaminoglycan metabolic process
B0030207biological_processchondroitin sulfate catabolic process
B0030209biological_processdermatan sulfate catabolic process
B0030214biological_processhyaluronan catabolic process
B0031410cellular_componentcytoplasmic vesicle
B0035578cellular_componentazurophil granule lumen
B0042552biological_processmyelination
B0042582cellular_componentazurophil granule
B0042802molecular_functionidentical protein binding
B0043202cellular_componentlysosomal lumen
B0043615biological_processastrocyte cell migration
B0045944biological_processpositive regulation of transcription by RNA polymerase II
B0048477biological_processoogenesis
B0050885biological_processneuromuscular process controlling balance
B0050905biological_processneuromuscular process
B0051651biological_processmaintenance of location in cell
B0060473cellular_componentcortical granule
B0070050biological_processneuron cellular homeostasis
B0070062cellular_componentextracellular exosome
B0102148molecular_functionN-acetyl-beta-D-galactosaminidase activity
B1905379cellular_componentbeta-N-acetylhexosaminidase complex
Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues2
DetailsACT_SITE: Proton donor => ECO:0000269|PubMed:11329289
ChainResidueDetails
AGLU355
BGLU355

site_idSWS_FT_FI2
Number of Residues2
DetailsSITE: Not glycosylated => ECO:0000269|PubMed:11447134
ChainResidueDetails
AASN497
BASN497

site_idSWS_FT_FI3
Number of Residues2
DetailsCARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:11447134, ECO:0000269|PubMed:19159218
ChainResidueDetails
AASN84
BASN84

site_idSWS_FT_FI4
Number of Residues4
DetailsCARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:11447134
ChainResidueDetails
AASN142
AASN190
BASN142
BASN190

site_idSWS_FT_FI5
Number of Residues2
DetailsCARBOHYD: N-linked (GlcNAc...) asparagine => ECO:0000269|PubMed:11447134, ECO:0000269|PubMed:12754519, ECO:0000269|PubMed:19159218
ChainResidueDetails
AASN327
BASN327

Catalytic Information from CSA
site_idCSA1
Number of Residues2
DetailsAnnotated By Reference To The Literature 1qba
ChainResidueDetails
AASP354
AGLU355

site_idCSA2
Number of Residues2
DetailsAnnotated By Reference To The Literature 1qba
ChainResidueDetails
BASP354
BGLU355

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PDB entries from 2024-07-24

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