1AUK

HUMAN ARYLSULFATASE A

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations
Protein Modification Annotation
Structure Motif: Primary M-CSA Annotation

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

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A	d1auka_	Alpha and beta proteins (a/b)	Alkaline phosphatase-like	Alkaline phosphatase-like	Arylsulfatase	Arylsulfatase A	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	SCOP2B Superfamily	Alkaline phosphatase-like	8032761	3001353	SCOP2B (2022-06-29)
A	SCOP2B Superfamily	Arylsulfatase C-terminal domain-like	8055769	3002305	SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	Sulfatase	e1aukA1	A: a/b three-layered sandwiches	X: Alkaline phosphatase-like (From Topology)	H: Alkaline phosphatase-like (From Topology)	T: Alkaline phosphatase-like	F: Sulfatase	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.40.720.10	Alpha Beta	3-Layer(aba) Sandwich	Alkaline Phosphatase, subunit A	Alkaline Phosphatase, subunit A	CATH (4.3.0)
A	3.30.1120.10	Alpha Beta	2-Layer Sandwich	Arylsulfatase, C-terminal domain		CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF14707	C-terminal region of aryl-sulfatase (Sulfatase_C)	C-terminal region of aryl-sulfatase	-	Domain
A	PF00884	Sulfatase (Sulfatase)	Sulfatase	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	ARYLSULFATASE A	cation binding ion binding binding calcium ion binding metal ion binding small molecule binding arylsulfatase activity hydrolase activity, acting on ester bonds hydrolase activity catalytic activity sulfuric ester hydrolase activity cerebroside-sulfatase activity	organic substance metabolic process primary metabolic process metabolic process lipid metabolic process	membrane-bounded organelle intracellular anatomical structure membrane-enclosed lumen cellular anatomical entity intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen endoplasmic reticulum lumen intracellular organelle organelle lumen organelle endoplasmic reticulum cytoplasm extracellular region membrane-bounded organelle intracellular anatomical structure membrane-enclosed lumen cellular anatomical entity intracellular membrane-bounded organelle endomembrane system intracellular organelle lumen endoplasmic reticulum lumen intracellular organelle organelle lumen organelle endoplasmic reticulum cytoplasm extracellular region lytic vacuole vacuolar lumen vacuole lysosomal lumen lysosome extracellular exosome extracellular vesicle extracellular organelle extracellular space vesicle extracellular membrane-bounded organelle intracellular vesicle cytoplasmic vesicle lumen cytoplasmic vesicle vesicle lumen azurophil granule secretory granule lumen azurophil granule lumen secretory granule primary lysosome secretory vesicle

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR024607	Sulfatase, conserved site	Conserved Site
A	IPR000917	Sulfatase, N-terminal	Domain
A	IPR017850	Alkaline-phosphatase-like, core domain superfamily	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: P15289	metachromatic leukodystrophy leukodystrophy peripheral neuropathy Unipolar Depression B-cell chronic lymphocytic leukemia inborn errors of metabolism lysosomal storage disease metachromatic leukodystrophy, juvenile form osteosarcoma X-linked mixed deafness with perilymphatic gusher diffuse large B-cell lymphoma intellectual disability major depressive disorder peripheral nervous system disease sphingolipidosis metachromatic leukodystrophy leukodystrophy peripheral neuropathy Unipolar Depression B-cell chronic lymphocytic leukemia inborn errors of metabolism lysosomal storage disease metachromatic leukodystrophy, juvenile form osteosarcoma X-linked mixed deafness with perilymphatic gusher diffuse large B-cell lymphoma intellectual disability major depressive disorder peripheral nervous system disease sphingolipidosis malignant mesothelioma mucosulfatidosis

Protein Modification Annotation

Modified Residue(s)
Chain	Residue(s)	Description
A	FGL	Parent Component: GLY RESID: AA0185 , AA0458 PSI-MOD : L-3-oxoalanine (Cys) MOD:00193 , L-3-oxoalanine (Ser) MOD:00835 , L-3-oxoalanine MOD:01169 , aminomalonic acid (Ser) MOD:01384

Structure Motif Annotation: Mechanism and Catalytic Site Atlas M-CSA Database Homepage

Chains	Enzyme Name	Description	Catalytic Residues
A	cerebroside-sulfatase M-CSA #158	Sulphatases are an evolutionarily highly conserved gene family. They hydrolyse sulphate ester bonds in a wide variety of structurally different compounds ranging from complex glucosaminoglucans and glycolipids to sulphated hydroxysteroids and amino acids. Among the sulphatase family, lysosomal arylsulphatase A (ASA) has been most extensively studied. The major physiological substrate of human ASA is a sphingolipid sulphate ester, cerebroside 3-sulphate, a major constituent of the myelin sheet. In vivo, the 3-sulphate group can be hydrolysed by ASA only if cerebroside 3-sulphate is complexed with the small activator protein saposin B solubilising the hydrophobic substrate.	Defined by 11 residues: ASP:A-11 [auth A-29]ASP:A-12 [auth A-30]GLY:A-51 [auth A-69]ARG:A-55 [auth A-73]LYS:A-105 [auth A-123]HIS:A-107 [auth A-125]SER:A-132 [auth A-150]HIS:A-211 [auth A-229]ASP:A-263 [auth A-281]ASN:A-264 [auth A-282]LYS:A-284 [auth A-302] \| Explore in 3D: M-CSA Motif Definition Up to 10 residues are supported for Structure Motif searching, this motif has 11 residues. EC: 3.1.6.8 (PDB Primary Data)

RCSB PDB Core Operations are funded by the National Science Foundation (DBI-1832184), 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 R01GM133198.