2DOS

Structural basis for the recognition of Lys48-linked polyubiquitin chain by the Josephin domain of ataxin-3, a putative deubiquitinating enzyme

External Resource: Annotation

Domain Annotation: ECOD Classification
Domain Annotation: CATH
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	Josephin	e2dosA1	A: a+b complex topology	X: Cysteine proteinases-like	H: Cysteine proteinases (From Topology)	T: Cysteine proteinases	F: Josephin	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.90.70.40	Alpha Beta	Alpha-Beta Complex	Cathepsin B	Chain A	CATH (4.3.0)
A	1.10.287.10	Mainly Alpha	Orthogonal Bundle	Helix Hairpins	S15/NS1, RNA-binding	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A	PF02099	Josephin (Josephin)	Josephin	-	Family

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	Ataxin-3	ubiquitin-like protein peptidase activity peptidase activity catalytic activity, acting on a protein hydrolase activity K48-linked deubiquitinase activity catalytic activity deubiquitinase activity K63-linked deubiquitinase activity cysteine-type peptidase activity cysteine-type deubiquitinase activity ubiquitin protein ligase binding protein binding binding ubiquitin-like protein ligase binding ubiquitin-like protein peptidase activity peptidase activity catalytic activity, acting on a protein hydrolase activity K48-linked deubiquitinase activity catalytic activity deubiquitinase activity K63-linked deubiquitinase activity cysteine-type peptidase activity cysteine-type deubiquitinase activity ubiquitin protein ligase binding protein binding binding ubiquitin-like protein ligase binding enzyme binding ATPase binding identical protein binding	behavior multicellular organismal process exploration behavior catabolic process positive regulation of protein metabolic process proteolysis involved in protein catabolic process positive regulation of biological process macromolecule catabolic process regulation of ubiquitin-dependent protein catabolic process regulation of primary metabolic process positive regulation of metabolic process organic substance metabolic process positive regulation of ubiquitin-dependent protein catabolic process regulation of proteolysis behavior multicellular organismal process exploration behavior catabolic process positive regulation of protein metabolic process proteolysis involved in protein catabolic process positive regulation of biological process macromolecule catabolic process regulation of ubiquitin-dependent protein catabolic process regulation of primary metabolic process positive regulation of metabolic process organic substance metabolic process positive regulation of ubiquitin-dependent protein catabolic process regulation of proteolysis regulation of proteolysis involved in protein catabolic process modification-dependent macromolecule catabolic process proteolysis positive regulation of macromolecule metabolic process protein metabolic process organonitrogen compound catabolic process regulation of biological process organonitrogen compound metabolic process regulation of protein metabolic process biological regulation primary metabolic process regulation of macromolecule metabolic process protein catabolic process regulation of catabolic process positive regulation of proteolysis involved in protein catabolic process positive regulation of catabolic process modification-dependent protein catabolic process positive regulation of proteolysis metabolic process ubiquitin-dependent protein catabolic process macromolecule metabolic process regulation of metabolic process organic substance catabolic process cellular response to stimulus cellular response to heat response to stimulus cellular response to stress response to stress response to heat response to abiotic stimulus cellular process response to temperature stimulus protein deubiquitination monoubiquitinated protein deubiquitination protein modification by small protein removal macromolecule modification post-translational protein modification protein modification process protein modification by small protein conjugation or removal cellular component organization organelle organization actin cytoskeleton organization actin filament-based process cellular component organization or biogenesis cytoskeleton organization protein K63-linked deubiquitination proteasomal protein catabolic process proteasome-mediated ubiquitin-dependent protein catabolic process protein localization to cytosolic proteasome complex protein localization localization cellular localization cellular macromolecule localization macromolecule localization developmental process multicellular organism development anatomical structure development nervous system development system development protein K48-linked deubiquitination trans-synaptic signaling synaptic signaling signaling anterograde trans-synaptic signaling cell-cell signaling chemical synaptic transmission cell communication microtubule cytoskeleton organization microtubule-based process regulation of cellular process negative regulation of signaling negative regulation of intracellular signal transduction negative regulation of biological process regulation of signal transduction intracellular signal transduction negative regulation of cell communication negative regulation of TORC1 signaling regulation of intracellular signal transduction negative regulation of TOR signaling negative regulation of cellular process signal transduction regulation of TORC1 signaling TOR signaling regulation of signaling regulation of TOR signaling negative regulation of response to stimulus negative regulation of signal transduction regulation of response to stimulus regulation of cell communication TORC1 signaling intermediate filament-based process intermediate filament cytoskeleton organization DNA metabolic process nucleic acid metabolic process nucleobase-containing compound metabolic process organic cyclic compound metabolic process nucleotide-excision repair DNA damage response DNA repair response to nutrient levels cellular response to amino acid starvation cellular response to starvation cellular response to nutrient levels response to amino acid starvation response to starvation response to organic substance cellular response to topologically incorrect protein cellular response to misfolded protein cellular response to chemical stimulus response to misfolded protein response to chemical response to topologically incorrect protein cellular response to organic substance protein quality control for misfolded or incompletely synthesized proteins positive regulation of proteasomal protein catabolic process regulation of cellular response to stress ERAD pathway response to endoplasmic reticulum stress regulation of ERAD pathway response to organonitrogen compound response to nitrogen compound positive regulation of protein catabolic process regulation of proteasomal protein catabolic process positive regulation of ERAD pathway regulation of response to endoplasmic reticulum stress positive regulation of response to stimulus positive regulation of response to endoplasmic reticulum stress regulation of response to stress positive regulation of cellular process regulation of protein catabolic process cell adhesion regulation of cell-substrate adhesion regulation of cell adhesion cell-substrate adhesion	membrane-bounded organelle intracellular anatomical structure nucleus membrane-enclosed lumen intracellular membrane-bounded organelle cellular anatomical entity intracellular organelle lumen intracellular organelle organelle lumen organelle nuclear periphery nuclear lumen nuclear matrix nucleolus membrane-bounded organelle intracellular anatomical structure nucleus membrane-enclosed lumen intracellular membrane-bounded organelle cellular anatomical entity intracellular organelle lumen intracellular organelle organelle lumen organelle nuclear periphery nuclear lumen nuclear matrix nucleolus intracellular non-membrane-bounded organelle non-membrane-bounded organelle mitochondrial membrane mitochondrion membrane organelle envelope mitochondrial envelope cytoplasm organelle membrane cytosol cell periphery plasma membrane nucleoplasm vacuolar membrane lytic vacuole vacuole lytic vacuole membrane bounding membrane of organelle lysosomal membrane lysosome synapse cell junction inclusion body nuclear inclusion body mitochondrial matrix

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR033865	Machado-Joseph disease protein	Family
A	IPR006155	Josephin domain	Domain
A	IPR003903	Ubiquitin interacting motif	Conserved Site

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: P54252	Machado-Joseph disease Nerve Degeneration autosomal dominant cerebellar ataxia depressive disorder intellectual disability psoriasis hereditary ataxia Kennedy disease neurodegenerative disease dentatorubral-pallidoluysian atrophy Huntington disease late-onset Parkinson disease Parkinson disease

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.