Summary Structural details Experimental details Functional details Sequence Neighbor History Downloads

2GJX

Crystallographic structure of human beta-Hexosaminidase A

Functional Information from GO Data

Chain	GOid	namespace	contents
A	0004553	molecular_function	hydrolase activity, hydrolyzing O-glycosyl compounds
A	0004563	molecular_function	beta-N-acetylhexosaminidase activity
A	0005515	molecular_function	protein binding
A	0005615	cellular_component	extracellular space
A	0005764	cellular_component	lysosome
A	0005975	biological_process	carbohydrate metabolic process
A	0006024	biological_process	glycosaminoglycan biosynthetic process
A	0006491	biological_process	N-glycan processing
A	0006629	biological_process	lipid metabolic process
A	0006689	biological_process	ganglioside catabolic process
A	0008375	molecular_function	acetylglucosaminyltransferase activity
A	0016020	cellular_component	membrane
A	0016787	molecular_function	hydrolase activity
A	0016798	molecular_function	hydrolase activity, acting on glycosyl bonds
A	0030203	biological_process	glycosaminoglycan metabolic process
A	0030209	biological_process	dermatan sulfate proteoglycan catabolic process
A	0030214	biological_process	hyaluronan catabolic process
A	0042582	cellular_component	azurophil granule
A	0043202	cellular_component	lysosomal lumen
A	0046982	molecular_function	protein heterodimerization activity
A	0070062	cellular_component	extracellular exosome
A	1901135	biological_process	carbohydrate derivative metabolic process
A	1905379	cellular_component	beta-N-acetylhexosaminidase complex
B	0001669	cellular_component	acrosomal vesicle
B	0004553	molecular_function	hydrolase activity, hydrolyzing O-glycosyl compounds
B	0004563	molecular_function	beta-N-acetylhexosaminidase activity
B	0005515	molecular_function	protein binding
B	0005576	cellular_component	extracellular region
B	0005615	cellular_component	extracellular space
B	0005764	cellular_component	lysosome
B	0005975	biological_process	carbohydrate metabolic process
B	0006044	biological_process	N-acetylglucosamine metabolic process
B	0006491	biological_process	N-glycan processing
B	0006629	biological_process	lipid metabolic process
B	0006689	biological_process	ganglioside catabolic process
B	0007338	biological_process	single fertilization
B	0008360	biological_process	regulation of cell shape
B	0008375	molecular_function	acetylglucosaminyltransferase activity
B	0015929	molecular_function	hexosaminidase activity
B	0016020	cellular_component	membrane
B	0016231	molecular_function	beta-N-acetylglucosaminidase activity
B	0016787	molecular_function	hydrolase activity
B	0016798	molecular_function	hydrolase activity, acting on glycosyl bonds
B	0030203	biological_process	glycosaminoglycan metabolic process
B	0030207	biological_process	chondroitin sulfate proteoglycan catabolic process
B	0030209	biological_process	dermatan sulfate proteoglycan catabolic process
B	0030214	biological_process	hyaluronan catabolic process
B	0030246	molecular_function	carbohydrate binding
B	0031410	cellular_component	cytoplasmic vesicle
B	0035578	cellular_component	azurophil granule lumen
B	0042582	cellular_component	azurophil granule
B	0042802	molecular_function	identical protein binding
B	0043202	cellular_component	lysosomal lumen
B	0043615	biological_process	astrocyte cell migration
B	0044877	molecular_function	protein-containing complex binding
B	0045944	biological_process	positive regulation of transcription by RNA polymerase II
B	0060473	cellular_component	cortical granule
B	0070062	cellular_component	extracellular exosome
B	1901135	biological_process	carbohydrate derivative metabolic process
B	1905379	cellular_component	beta-N-acetylhexosaminidase complex
C	0001669	cellular_component	acrosomal vesicle
C	0004553	molecular_function	hydrolase activity, hydrolyzing O-glycosyl compounds
C	0004563	molecular_function	beta-N-acetylhexosaminidase activity
C	0005515	molecular_function	protein binding
C	0005576	cellular_component	extracellular region
C	0005615	cellular_component	extracellular space
C	0005764	cellular_component	lysosome
C	0005975	biological_process	carbohydrate metabolic process
C	0006044	biological_process	N-acetylglucosamine metabolic process
C	0006491	biological_process	N-glycan processing
C	0006629	biological_process	lipid metabolic process
C	0006689	biological_process	ganglioside catabolic process
C	0007338	biological_process	single fertilization
C	0008360	biological_process	regulation of cell shape
C	0008375	molecular_function	acetylglucosaminyltransferase activity
C	0015929	molecular_function	hexosaminidase activity
C	0016020	cellular_component	membrane
C	0016231	molecular_function	beta-N-acetylglucosaminidase activity
C	0016787	molecular_function	hydrolase activity
C	0016798	molecular_function	hydrolase activity, acting on glycosyl bonds
C	0030203	biological_process	glycosaminoglycan metabolic process
C	0030207	biological_process	chondroitin sulfate proteoglycan catabolic process
C	0030209	biological_process	dermatan sulfate proteoglycan catabolic process
C	0030214	biological_process	hyaluronan catabolic process
C	0030246	molecular_function	carbohydrate binding
C	0031410	cellular_component	cytoplasmic vesicle
C	0035578	cellular_component	azurophil granule lumen
C	0042582	cellular_component	azurophil granule
C	0042802	molecular_function	identical protein binding
C	0043202	cellular_component	lysosomal lumen
C	0043615	biological_process	astrocyte cell migration
C	0044877	molecular_function	protein-containing complex binding
C	0045944	biological_process	positive regulation of transcription by RNA polymerase II
C	0060473	cellular_component	cortical granule
C	0070062	cellular_component	extracellular exosome
C	1901135	biological_process	carbohydrate derivative metabolic process
C	1905379	cellular_component	beta-N-acetylhexosaminidase complex
D	0004553	molecular_function	hydrolase activity, hydrolyzing O-glycosyl compounds
D	0004563	molecular_function	beta-N-acetylhexosaminidase activity
D	0005515	molecular_function	protein binding
D	0005615	cellular_component	extracellular space
D	0005764	cellular_component	lysosome
D	0005975	biological_process	carbohydrate metabolic process
D	0006024	biological_process	glycosaminoglycan biosynthetic process
D	0006491	biological_process	N-glycan processing
D	0006629	biological_process	lipid metabolic process
D	0006689	biological_process	ganglioside catabolic process
D	0008375	molecular_function	acetylglucosaminyltransferase activity
D	0016020	cellular_component	membrane
D	0016787	molecular_function	hydrolase activity
D	0016798	molecular_function	hydrolase activity, acting on glycosyl bonds
D	0030203	biological_process	glycosaminoglycan metabolic process
D	0030209	biological_process	dermatan sulfate proteoglycan catabolic process
D	0030214	biological_process	hyaluronan catabolic process
D	0042582	cellular_component	azurophil granule
D	0043202	cellular_component	lysosomal lumen
D	0046982	molecular_function	protein heterodimerization activity
D	0070062	cellular_component	extracellular exosome
D	1901135	biological_process	carbohydrate derivative metabolic process
D	1905379	cellular_component	beta-N-acetylhexosaminidase complex
E	0004553	molecular_function	hydrolase activity, hydrolyzing O-glycosyl compounds
E	0004563	molecular_function	beta-N-acetylhexosaminidase activity
E	0005515	molecular_function	protein binding
E	0005615	cellular_component	extracellular space
E	0005764	cellular_component	lysosome
E	0005975	biological_process	carbohydrate metabolic process
E	0006024	biological_process	glycosaminoglycan biosynthetic process
E	0006491	biological_process	N-glycan processing
E	0006629	biological_process	lipid metabolic process
E	0006689	biological_process	ganglioside catabolic process
E	0008375	molecular_function	acetylglucosaminyltransferase activity
E	0016020	cellular_component	membrane
E	0016787	molecular_function	hydrolase activity
E	0016798	molecular_function	hydrolase activity, acting on glycosyl bonds
E	0030203	biological_process	glycosaminoglycan metabolic process
E	0030209	biological_process	dermatan sulfate proteoglycan catabolic process
E	0030214	biological_process	hyaluronan catabolic process
E	0042582	cellular_component	azurophil granule
E	0043202	cellular_component	lysosomal lumen
E	0046982	molecular_function	protein heterodimerization activity
E	0070062	cellular_component	extracellular exosome
E	1901135	biological_process	carbohydrate derivative metabolic process
E	1905379	cellular_component	beta-N-acetylhexosaminidase complex
F	0001669	cellular_component	acrosomal vesicle
F	0004553	molecular_function	hydrolase activity, hydrolyzing O-glycosyl compounds
F	0004563	molecular_function	beta-N-acetylhexosaminidase activity
F	0005515	molecular_function	protein binding
F	0005576	cellular_component	extracellular region
F	0005615	cellular_component	extracellular space
F	0005764	cellular_component	lysosome
F	0005975	biological_process	carbohydrate metabolic process
F	0006044	biological_process	N-acetylglucosamine metabolic process
F	0006491	biological_process	N-glycan processing
F	0006629	biological_process	lipid metabolic process
F	0006689	biological_process	ganglioside catabolic process
F	0007338	biological_process	single fertilization
F	0008360	biological_process	regulation of cell shape
F	0008375	molecular_function	acetylglucosaminyltransferase activity
F	0015929	molecular_function	hexosaminidase activity
F	0016020	cellular_component	membrane
F	0016231	molecular_function	beta-N-acetylglucosaminidase activity
F	0016787	molecular_function	hydrolase activity
F	0016798	molecular_function	hydrolase activity, acting on glycosyl bonds
F	0030203	biological_process	glycosaminoglycan metabolic process
F	0030207	biological_process	chondroitin sulfate proteoglycan catabolic process
F	0030209	biological_process	dermatan sulfate proteoglycan catabolic process
F	0030214	biological_process	hyaluronan catabolic process
F	0030246	molecular_function	carbohydrate binding
F	0031410	cellular_component	cytoplasmic vesicle
F	0035578	cellular_component	azurophil granule lumen
F	0042582	cellular_component	azurophil granule
F	0042802	molecular_function	identical protein binding
F	0043202	cellular_component	lysosomal lumen
F	0043615	biological_process	astrocyte cell migration
F	0044877	molecular_function	protein-containing complex binding
F	0045944	biological_process	positive regulation of transcription by RNA polymerase II
F	0060473	cellular_component	cortical granule
F	0070062	cellular_component	extracellular exosome
F	1901135	biological_process	carbohydrate derivative metabolic process
F	1905379	cellular_component	beta-N-acetylhexosaminidase complex
G	0001669	cellular_component	acrosomal vesicle
G	0004553	molecular_function	hydrolase activity, hydrolyzing O-glycosyl compounds
G	0004563	molecular_function	beta-N-acetylhexosaminidase activity
G	0005515	molecular_function	protein binding
G	0005576	cellular_component	extracellular region
G	0005615	cellular_component	extracellular space
G	0005764	cellular_component	lysosome
G	0005975	biological_process	carbohydrate metabolic process
G	0006044	biological_process	N-acetylglucosamine metabolic process
G	0006491	biological_process	N-glycan processing
G	0006629	biological_process	lipid metabolic process
G	0006689	biological_process	ganglioside catabolic process
G	0007338	biological_process	single fertilization
G	0008360	biological_process	regulation of cell shape
G	0008375	molecular_function	acetylglucosaminyltransferase activity
G	0015929	molecular_function	hexosaminidase activity
G	0016020	cellular_component	membrane
G	0016231	molecular_function	beta-N-acetylglucosaminidase activity
G	0016787	molecular_function	hydrolase activity
G	0016798	molecular_function	hydrolase activity, acting on glycosyl bonds
G	0030203	biological_process	glycosaminoglycan metabolic process
G	0030207	biological_process	chondroitin sulfate proteoglycan catabolic process
G	0030209	biological_process	dermatan sulfate proteoglycan catabolic process
G	0030214	biological_process	hyaluronan catabolic process
G	0030246	molecular_function	carbohydrate binding
G	0031410	cellular_component	cytoplasmic vesicle
G	0035578	cellular_component	azurophil granule lumen
G	0042582	cellular_component	azurophil granule
G	0042802	molecular_function	identical protein binding
G	0043202	cellular_component	lysosomal lumen
G	0043615	biological_process	astrocyte cell migration
G	0044877	molecular_function	protein-containing complex binding
G	0045944	biological_process	positive regulation of transcription by RNA polymerase II
G	0060473	cellular_component	cortical granule
G	0070062	cellular_component	extracellular exosome
G	1901135	biological_process	carbohydrate derivative metabolic process
G	1905379	cellular_component	beta-N-acetylhexosaminidase complex
H	0004553	molecular_function	hydrolase activity, hydrolyzing O-glycosyl compounds
H	0004563	molecular_function	beta-N-acetylhexosaminidase activity
H	0005515	molecular_function	protein binding
H	0005615	cellular_component	extracellular space
H	0005764	cellular_component	lysosome
H	0005975	biological_process	carbohydrate metabolic process
H	0006024	biological_process	glycosaminoglycan biosynthetic process
H	0006491	biological_process	N-glycan processing
H	0006629	biological_process	lipid metabolic process
H	0006689	biological_process	ganglioside catabolic process
H	0008375	molecular_function	acetylglucosaminyltransferase activity
H	0016020	cellular_component	membrane
H	0016787	molecular_function	hydrolase activity
H	0016798	molecular_function	hydrolase activity, acting on glycosyl bonds
H	0030203	biological_process	glycosaminoglycan metabolic process
H	0030209	biological_process	dermatan sulfate proteoglycan catabolic process
H	0030214	biological_process	hyaluronan catabolic process
H	0042582	cellular_component	azurophil granule
H	0043202	cellular_component	lysosomal lumen
H	0046982	molecular_function	protein heterodimerization activity
H	0070062	cellular_component	extracellular exosome
H	1901135	biological_process	carbohydrate derivative metabolic process
H	1905379	cellular_component	beta-N-acetylhexosaminidase complex

Functional Information from SwissProt/UniProt

site_id	SWS_FT_FI1
Number of Residues	4
Details	Region: {"description":"Critical for hydrolysis GM2 gangliosides"}

Chain

Residue

Details

site_id	SWS_FT_FI2
Number of Residues	4
Details	Active site: {"description":"Proton donor","evidences":[{"source":"PubMed","id":"16698036","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

site_id	SWS_FT_FI3
Number of Residues	4
Details	Glycosylation: {"description":"N-linked (GlcNAc...) asparagine","evidences":[{"source":"PubMed","id":"1533633","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"16698036","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"11707436","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

site_id	SWS_FT_FI4
Number of Residues	8
Details	Glycosylation: {"description":"N-linked (GlcNAc...) asparagine","evidences":[{"source":"PubMed","id":"1533633","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"16698036","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"19159218","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"11707436","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

site_id	SWS_FT_FI5
Number of Residues	4
Details	Active site: {"description":"Proton donor","evidences":[{"source":"PubMed","id":"11329289","evidenceCode":"ECO:0000269"}]}

Chain

Residue

Details

site_id	SWS_FT_FI6
Number of Residues	4
Details	Site: {"description":"Not glycosylated","evidences":[{"source":"PubMed","id":"11447134","evidenceCode":"ECO:0000269"}]}

Chain

Residue

Details

site_id	SWS_FT_FI7
Number of Residues	4
Details	Glycosylation: {"description":"N-linked (GlcNAc...) asparagine","evidences":[{"source":"PubMed","id":"11447134","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"19159218","evidenceCode":"ECO:0000269"}]}

Chain

Residue

Details

site_id	SWS_FT_FI8
Number of Residues	8
Details	Glycosylation: {"description":"N-linked (GlcNAc...) asparagine","evidences":[{"source":"PubMed","id":"11447134","evidenceCode":"ECO:0000269"}]}

Chain

Residue

Details

site_id	SWS_FT_FI9
Number of Residues	4
Details	Glycosylation: {"description":"N-linked (GlcNAc...) asparagine","evidences":[{"source":"PubMed","id":"11447134","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"12754519","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"19159218","evidenceCode":"ECO:0000269"}]}

Chain

Residue

Details

Catalytic Information from CSA

site_id	CSA1
Number of Residues	2
Details	Annotated By Reference To The Literature 1qba

Chain	Residue	Details
B	ASP354
B	GLU355

site_id	CSA2
Number of Residues	2
Details	Annotated By Reference To The Literature 1qba

Chain	Residue	Details
C	ASP354
C	GLU355

site_id	CSA3
Number of Residues	2
Details	Annotated By Reference To The Literature 1qba

Chain	Residue	Details
F	ASP354
F	GLU355

site_id	CSA4
Number of Residues	2
Details	Annotated By Reference To The Literature 1qba

Chain	Residue	Details
G	ASP354
G	GLU355

site_id	CSA5
Number of Residues	2
Details	Annotated By Reference To The Literature 1qba

Chain	Residue	Details
A	GLU323
A	ASP322

site_id	CSA6
Number of Residues	2
Details	Annotated By Reference To The Literature 1qba

Chain	Residue	Details
D	GLU323
D	ASP322

site_id	CSA7
Number of Residues	2
Details	Annotated By Reference To The Literature 1qba

Chain	Residue	Details
E	GLU323
E	ASP322

site_id	CSA8
Number of Residues	2
Details	Annotated By Reference To The Literature 1qba

Chain	Residue	Details
H	GLU323
H	ASP322

		Sequence (fasta)
		PDBx/mmCIF
		PDBML format (no-atom)
		PDB format (full)
		Validation report (PDF)
		EDMap 2fo-fc (MTZ)