4DO4

Pharmacological chaperones for human alpha-N-acetylgalactosaminidase

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: SCOP/SCOPe Classification SCOP-e Database Homepage

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A	d4do4a2	All beta proteins	Glycosyl hydrolase domain	Glycosyl hydrolase domain	automated matches	automated matches	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d4do4a1	Alpha and beta proteins (a/b)	TIM beta/alpha-barrel	(Trans)glycosidases	Amylase, catalytic domain	automated matches	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d4do4b2	All beta proteins	Glycosyl hydrolase domain	Glycosyl hydrolase domain	automated matches	automated matches	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d4do4b1	Alpha and beta proteins (a/b)	TIM beta/alpha-barrel	(Trans)glycosidases	Amylase, catalytic domain	automated matches	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d4do4b3	Artifacts	Tags	Tags	Tags	C-terminal Tags	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	Melibiase_2_C_1	e4do4A2	A: beta sandwiches	X: Glycosyl hydrolase domain-like	H: Glycosyl hydrolase domain (From Topology)	T: Glycosyl hydrolase domain	F: Melibiase_2_C_1	ECOD (1.6)
A	Glyco_hydro_31_N	e4do4A1	A: a/b barrels	X: TIM beta/alpha-barrel	H: TIM barrels (From Topology)	T: TIM barrels	F: Glyco_hydro_31_N	ECOD (1.6)
B	Melibiase_2_C_1	e4do4B1	A: beta sandwiches	X: Glycosyl hydrolase domain-like	H: Glycosyl hydrolase domain (From Topology)	T: Glycosyl hydrolase domain	F: Melibiase_2_C_1	ECOD (1.6)
B	Glyco_hydro_31_N	e4do4B2	A: a/b barrels	X: TIM beta/alpha-barrel	H: TIM barrels (From Topology)	T: TIM barrels	F: Glyco_hydro_31_N	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.20.20.70	Alpha Beta	Alpha-Beta Barrel	TIM Barrel	Aldolase class I	CATH (4.3.0)
A	2.60.40.1180	Mainly Beta	Sandwich	Immunoglobulin-like	Golgi alpha-mannosidase II	CATH (4.3.0)
B	3.20.20.70	Alpha Beta	Alpha-Beta Barrel	TIM Barrel	Aldolase class I	CATH (4.3.0)
B	2.60.40.1180	Mainly Beta	Sandwich	Immunoglobulin-like	Golgi alpha-mannosidase II	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B	PF17450	Alpha galactosidase A C-terminal beta sandwich domain (Melibiase_2_C)	Alpha galactosidase A C-terminal beta sandwich domain	-	Domain
A, B	PF16499	Alpha galactosidase A (Melibiase_2)	Alpha galactosidase A	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B	Alpha-N-acetylgalactosaminidase	alpha-galactosidase activity hydrolase activity, acting on glycosyl bonds hydrolase activity, hydrolyzing O-glycosyl compounds hydrolase activity catalytic activity galactosidase activity identical protein binding protein binding binding protein homodimerization activity protein dimerization activity hexosaminidase activity alpha-N-acetylgalactosaminidase activity	carbohydrate metabolic process oligosaccharide metabolic process primary metabolic process metabolic process glycoside catabolic process carbohydrate derivative catabolic process glycosyl compound metabolic process catabolic process glycoside metabolic process carbohydrate derivative metabolic process glycosyl compound catabolic process carbohydrate catabolic process membrane lipid metabolic process lipid catabolic process carbohydrate metabolic process oligosaccharide metabolic process primary metabolic process metabolic process glycoside catabolic process carbohydrate derivative catabolic process glycosyl compound metabolic process catabolic process glycoside metabolic process carbohydrate derivative metabolic process glycosyl compound catabolic process carbohydrate catabolic process membrane lipid metabolic process lipid catabolic process glycolipid metabolic process cellular metabolic process liposaccharide metabolic process membrane lipid catabolic process cellular lipid catabolic process cellular catabolic process glycolipid catabolic process cellular lipid metabolic process cellular process lipid metabolic process	extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space cellular anatomical entity vesicle extracellular membrane-bounded organelle intracellular anatomical structure cytoplasm intracellular organelle lytic vacuole extracellular exosome membrane-bounded organelle extracellular vesicle extracellular organelle extracellular region organelle extracellular space cellular anatomical entity vesicle extracellular membrane-bounded organelle intracellular anatomical structure cytoplasm intracellular organelle lytic vacuole vacuole intracellular membrane-bounded organelle lysosome

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B	IPR002241	Glycoside hydrolase, family 27	Family
A, B	IPR017853	Glycoside hydrolase superfamily	Homologous Superfamily
A, B	IPR013785	Aldolase-type TIM barrel	Homologous Superfamily
A, B	IPR000111	Glycoside hydrolase family 27/36, conserved site	Conserved Site
A, B	IPR035373	Alpha galactosidase A, C-terminal beta-sandwich domain	Domain
A, B	IPR013780	Glycosyl hydrolase, all-beta	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B	Pharos: P17050	alpha-N-acetylgalactosaminidase deficiency type 2 neuroaxonal dystrophy Schindler disease alpha-N-acetylgalactosaminidase deficiency type 3 alpha-N-acetylgalactosaminidase deficiency type 1 tuberculosis epilepsy glioblastoma intellectual disability malignant ependymoma osteosarcoma astrocytic tumor malignant mesothelioma pilocytic astrocytoma subependymal giant cell astrocytoma alpha-N-acetylgalactosaminidase deficiency type 2 neuroaxonal dystrophy Schindler disease alpha-N-acetylgalactosaminidase deficiency type 3 alpha-N-acetylgalactosaminidase deficiency type 1 tuberculosis epilepsy glioblastoma intellectual disability malignant ependymoma osteosarcoma astrocytic tumor malignant mesothelioma pilocytic astrocytoma subependymal giant cell astrocytoma autism spectrum disorder

RCSB PDB Core Operations are funded by the U.S. National Science Foundation (DBI-2321666), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM157729.