4MJ2

Crystal structure of apo-iduronidase in the R3 form

External Resource: Annotation

Domain Annotation: ECOD Classification
Domain Annotation: CATH
IMGT Antibody Annotation
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	EUF07725	e4mj2A5	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: EUF07725	ECOD (1.6)
A	Glyco_hydro_39_2nd	e4mj2A2	A: beta sandwiches	X: Glycosyl hydrolase domain-like	H: Glycosyl hydrolase domain (From Topology)	T: Glycosyl hydrolase domain	F: Glyco_hydro_39_2nd	ECOD (1.6)
A	Glyco_hydro_3	e4mj2A1	A: a/b barrels	X: TIM beta/alpha-barrel	H: TIM barrels (From Topology)	T: TIM barrels	F: Glyco_hydro_3	ECOD (1.6)
B	EUF07725	e4mj2B12	A: beta sandwiches	X: Immunoglobulin-like beta-sandwich	H: Immunoglobulin-related	T: Immunoglobulin/Fibronectin type III/E set domains/PapD-like	F: EUF07725	ECOD (1.6)
B	Glyco_hydro_39_2nd	e4mj2B11	A: beta sandwiches	X: Glycosyl hydrolase domain-like	H: Glycosyl hydrolase domain (From Topology)	T: Glycosyl hydrolase domain	F: Glyco_hydro_39_2nd	ECOD (1.6)
B	Glyco_hydro_3	e4mj2B10	A: a/b barrels	X: TIM beta/alpha-barrel	H: TIM barrels (From Topology)	T: TIM barrels	F: Glyco_hydro_3	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	2.60.40.1500	Mainly Beta	Sandwich	Immunoglobulin-like	Glycosyl hydrolase domain	CATH (4.3.0)
A	3.20.20.80	Alpha Beta	Alpha-Beta Barrel	TIM Barrel	Glycosidases	CATH (4.3.0)
A	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (4.3.0)
B	2.60.40.1500	Mainly Beta	Sandwich	Immunoglobulin-like	Glycosyl hydrolase domain	CATH (4.3.0)
B	3.20.20.80	Alpha Beta	Alpha-Beta Barrel	TIM Barrel	Glycosidases	CATH (4.3.0)
B	2.60.40.10	Mainly Beta	Sandwich	Immunoglobulin-like	Immunoglobulins	CATH (4.3.0)

IMGT Antibody Annotation IMGT Database Homepage

Chain	Protein Name	Description	Organism Name	Gene Allele Name(s)	Domain Name(s)	Receptor Type	Receptor Description	Provenance Source
A, B	Apo iduronidase	IDUA	Homo sapiens (human)	IDUA*01	R-LIKE-DOMAIN IDUA (Pr27-653)	RPI	IDUA	IMGT (202448-0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B	PF21200	Alpha-L-iduronidase C-terminal domain (Glyco_hydro_39_C)	Alpha-L-iduronidase C-terminal domain	This entry represents the C-terminal Ig-fold domain of Alpha-L-iduronidase enzyme [1-4].	Domain
A, B	PF01229	Glycosyl hydrolases family 39 (Glyco_hydro_39)	Glycosyl hydrolases family 39	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B	Alpha-L-iduronidase	protein binding binding signaling receptor binding hydrolase activity, acting on glycosyl bonds hydrolase activity, hydrolyzing O-glycosyl compounds hydrolase activity catalytic activity L-iduronidase activity	carbohydrate derivative catabolic process catabolic process glycosaminoglycan metabolic process carbohydrate derivative metabolic process aminoglycan catabolic process macromolecule catabolic process glycosaminoglycan catabolic process aminoglycan metabolic process metabolic process cellular process macromolecule metabolic process mucopolysaccharide metabolic process dermatan sulfate proteoglycan metabolic process oxoacid metabolic process carbohydrate derivative catabolic process catabolic process glycosaminoglycan metabolic process carbohydrate derivative metabolic process aminoglycan catabolic process macromolecule catabolic process glycosaminoglycan catabolic process aminoglycan metabolic process metabolic process cellular process macromolecule metabolic process mucopolysaccharide metabolic process dermatan sulfate proteoglycan metabolic process oxoacid metabolic process organic acid metabolic process organic acid catabolic process cellular catabolic process monocarboxylic acid catabolic process carboxylic acid catabolic process sulfur compound metabolic process small molecule metabolic process protein metabolic process carboxylic acid metabolic process primary metabolic process glycoprotein metabolic process dermatan sulfate catabolic process sulfur compound catabolic process dermatan sulfate metabolic process monocarboxylic acid metabolic process proteoglycan metabolic process small molecule catabolic process glycoprotein catabolic process protein catabolic process heparan sulfate proteoglycan catabolic process proteoglycan catabolic process carbohydrate metabolic process oligosaccharide metabolic process disaccharide metabolic process heparin metabolic process heparin catabolic process	membrane-bounded organelle intracellular anatomical structure lytic vacuole membrane-enclosed lumen vacuolar lumen vacuole intracellular membrane-bounded organelle lysosomal lumen intracellular organelle lumen intracellular organelle cellular anatomical structure organelle lumen organelle cytoplasm membrane-bounded organelle intracellular anatomical structure lytic vacuole membrane-enclosed lumen vacuolar lumen vacuole intracellular membrane-bounded organelle lysosomal lumen intracellular organelle lumen intracellular organelle cellular anatomical structure organelle lumen organelle cytoplasm lysosome extracellular exosome extracellular vesicle extracellular organelle extracellular region extracellular space vesicle extracellular membrane-bounded organelle

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B	IPR000514	Glycoside hydrolase, family 39	Family
A, B	IPR049167	Alpha-L-iduronidase, C-terminal domain	Domain
A, B	IPR017853	Glycoside hydrolase superfamily	Homologous Superfamily
A, B	IPR049166	Glycosyl hydrolases family 39, N-terminal catalytic domain	Domain
A, B	IPR051923	Glycosyl Hydrolase Family 39	Family
A, B	IPR013783	Immunoglobulin-like fold	Homologous Superfamily
A, B	IPR049165	Glycosyl hydrolases family 39, active site	Active Site

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B	Pharos: P35475	Scheie syndrome Hurler-Scheie syndrome mucopolysaccharidosis type 1 mucopolysaccharidosis Hurler syndrome alpha-L-Iduronidase Deficiency lysosomal storage disease nephrolithiasis susceptibility caused by SLC26A1 primary biliary cholangitis systemic lupus erythematosus bone disease inborn errors of metabolism malignant mesothelioma rheumatoid arthritis systemic sclerosis Scheie syndrome Hurler-Scheie syndrome mucopolysaccharidosis type 1 mucopolysaccharidosis Hurler syndrome alpha-L-Iduronidase Deficiency lysosomal storage disease nephrolithiasis susceptibility caused by SLC26A1 primary biliary cholangitis systemic lupus erythematosus bone disease inborn errors of metabolism malignant mesothelioma rheumatoid arthritis systemic sclerosis craniosynostosis hydrops fetalis intellectual disability myositis plasma cell myeloma

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.