2JA3

Cytoplasmic Domain of the Human Chloride Transporter ClC-5 in complex with ADP

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

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

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A	d2ja3a1	Alpha and beta proteins (a+b)	CBS-domain pair	CBS-domain pair	CBS-domain pair	automated matches	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d2ja3a2	Artifacts	Tags	Tags	Tags	C-terminal Tags	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d2ja3b1	Alpha and beta proteins (a+b)	CBS-domain pair	CBS-domain pair	CBS-domain pair	automated matches	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d2ja3b2	Artifacts	Tags	Tags	Tags	C-terminal Tags	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
C	d2ja3c1	Alpha and beta proteins (a+b)	CBS-domain pair	CBS-domain pair	CBS-domain pair	automated matches	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
C	d2ja3c2	Artifacts	Tags	Tags	Tags	C-terminal Tags	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
D	d2ja3d1	Alpha and beta proteins (a+b)	CBS-domain pair	CBS-domain pair	CBS-domain pair	automated matches	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
D	d2ja3d2	Artifacts	Tags	Tags	Tags	C-terminal Tags	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
E	d2ja3e1	Alpha and beta proteins (a+b)	CBS-domain pair	CBS-domain pair	CBS-domain pair	automated matches	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
E	d2ja3e2	Artifacts	Tags	Tags	Tags	C-terminal Tags	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
F	d2ja3f1	Alpha and beta proteins (a+b)	CBS-domain pair	CBS-domain pair	CBS-domain pair	automated matches	HUMAN (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
F	d2ja3f2	Artifacts	Tags	Tags	Tags	C-terminal Tags	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	CBS-domain pair	8034653	3000123	SCOP2B (2022-06-29)
B	SCOP2B Superfamily	CBS-domain pair	8034653	3000123	SCOP2B (2022-06-29)
C	SCOP2B Superfamily	CBS-domain pair	8034653	3000123	SCOP2B (2022-06-29)
D	SCOP2B Superfamily	CBS-domain pair	8034653	3000123	SCOP2B (2022-06-29)
E	SCOP2B Superfamily	CBS-domain pair	8034653	3000123	SCOP2B (2022-06-29)
F	SCOP2B Superfamily	CBS-domain pair	8034653	3000123	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	IMPDH_2nd_1	e2ja3A1	A: a+b duplicates or obligate multimers	X: CBS-domain (From Topology)	H: CBS-domain (From Topology)	T: CBS-domain	F: IMPDH_2nd_1	ECOD (1.6)
B	IMPDH_2nd_1	e2ja3B1	A: a+b duplicates or obligate multimers	X: CBS-domain (From Topology)	H: CBS-domain (From Topology)	T: CBS-domain	F: IMPDH_2nd_1	ECOD (1.6)
C	IMPDH_2nd_1	e2ja3C1	A: a+b duplicates or obligate multimers	X: CBS-domain (From Topology)	H: CBS-domain (From Topology)	T: CBS-domain	F: IMPDH_2nd_1	ECOD (1.6)
D	IMPDH_2nd_1	e2ja3D1	A: a+b duplicates or obligate multimers	X: CBS-domain (From Topology)	H: CBS-domain (From Topology)	T: CBS-domain	F: IMPDH_2nd_1	ECOD (1.6)
E	IMPDH_2nd_1	e2ja3E1	A: a+b duplicates or obligate multimers	X: CBS-domain (From Topology)	H: CBS-domain (From Topology)	T: CBS-domain	F: IMPDH_2nd_1	ECOD (1.6)
F	IMPDH_2nd_1	e2ja3F1	A: a+b duplicates or obligate multimers	X: CBS-domain (From Topology)	H: CBS-domain (From Topology)	T: CBS-domain	F: IMPDH_2nd_1	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.90.1280.20	Alpha Beta	Alpha-Beta Complex	CBS domain Like		CATH (4.3.0)
A	3.10.580.20	Alpha Beta	Roll	CBS-domain		CATH (4.3.0)
B	3.90.1280.20	Alpha Beta	Alpha-Beta Complex	CBS domain Like		CATH (4.3.0)
B	3.10.580.20	Alpha Beta	Roll	CBS-domain		CATH (4.3.0)
C	3.90.1280.20	Alpha Beta	Alpha-Beta Complex	CBS domain Like		CATH (4.3.0)
C	3.10.580.20	Alpha Beta	Roll	CBS-domain		CATH (4.3.0)
D	3.90.1280.20	Alpha Beta	Alpha-Beta Complex	CBS domain Like		CATH (4.3.0)
D	3.10.580.20	Alpha Beta	Roll	CBS-domain		CATH (4.3.0)
E	3.90.1280.20	Alpha Beta	Alpha-Beta Complex	CBS domain Like		CATH (4.3.0)
E	3.10.580.20	Alpha Beta	Roll	CBS-domain		CATH (4.3.0)
F	3.90.1280.20	Alpha Beta	Alpha-Beta Complex	CBS domain Like		CATH (4.3.0)
F	3.10.580.20	Alpha Beta	Roll	CBS-domain		CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B, C, D, E A, B, C, D, E, F	PF00571	CBS domain (CBS)	CBS domain	CBS domains are small intracellular modules that pair together to form a stable globular domain [2]. This family represents a single CBS domain. Pairs of these domains have been termed a Bateman domain [6]. CBS domains have been shown to bind ligands ...	CBS domains are small intracellular modules that pair together to form a stable globular domain [2]. This family represents a single CBS domain. Pairs of these domains have been termed a Bateman domain [6]. CBS domains have been shown to bind ligands with an adenosyl group such as AMP, ATP and S-AdoMet [5]. CBS domains are found attached to a wide range of other protein domains suggesting that CBS domains may play a regulatory role making proteins sensitive to adenosyl carrying ligands. The region containing the CBS domains in Cystathionine-beta synthase is involved in regulation by S-AdoMet [4]. CBS domain pairs from AMPK bind AMP or ATP [5]. The CBS domains from IMPDH and the chloride channel CLC2 bind ATP [5].	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B, C, D, E A, B, C, D, E, F	CHLORIDE CHANNEL PROTEIN 5	binding adenyl ribonucleotide binding nucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding ion binding purine nucleotide binding organic cyclic compound binding anion binding adenyl nucleotide binding ATP binding heterocyclic compound binding carbohydrate derivative binding binding adenyl ribonucleotide binding nucleotide binding purine ribonucleotide binding purine ribonucleoside triphosphate binding nucleoside phosphate binding ion binding purine nucleotide binding organic cyclic compound binding anion binding adenyl nucleotide binding ATP binding heterocyclic compound binding carbohydrate derivative binding ribonucleotide binding small molecule binding voltage-gated chloride channel activity monoatomic anion transmembrane transporter activity chloride transmembrane transporter activity gated channel activity monoatomic ion channel activity voltage-gated channel activity voltage-gated monoatomic ion channel activity voltage-gated monoatomic anion channel activity passive transmembrane transporter activity inorganic molecular entity transmembrane transporter activity channel activity monoatomic anion channel activity monoatomic ion transmembrane transporter activity transporter activity transmembrane transporter activity inorganic anion transmembrane transporter activity chloride channel activity antiporter activity active transmembrane transporter activity secondary active transmembrane transporter activity identical protein binding protein binding	localization transport monoatomic anion transport monoatomic ion transport chloride transport establishment of localization inorganic anion transport multicellular organismal process renal system process system process import into cell cellular process endocytosis vesicle-mediated transport localization transport monoatomic anion transport monoatomic ion transport chloride transport establishment of localization inorganic anion transport multicellular organismal process renal system process system process import into cell cellular process endocytosis vesicle-mediated transport transmembrane transport monoatomic ion transmembrane transport	membrane-bounded organelle intracellular anatomical structure early endosome endosome intracellular vesicle cellular anatomical entity intracellular membrane-bounded organelle endomembrane system intracellular organelle cytoplasmic vesicle organelle cytoplasm vesicle vacuolar membrane membrane-bounded organelle intracellular anatomical structure early endosome endosome intracellular vesicle cellular anatomical entity intracellular membrane-bounded organelle endomembrane system intracellular organelle cytoplasmic vesicle organelle cytoplasm vesicle vacuolar membrane membrane lytic vacuole vacuole lytic vacuole membrane bounding membrane of organelle lysosomal membrane organelle membrane lysosome apical part of cell Golgi apparatus Golgi membrane cytosol endosome membrane cytoplasmic vesicle membrane vesicle membrane cell periphery plasma membrane transport vesicle synapse presynapse synaptic vesicle cell junction secretory vesicle exocytic vesicle

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B, C, D, E A, B, C, D, E, F	IPR046342	CBS domain superfamily	Homologous Superfamily
A, B, C, D, E A, B, C, D, E, F	IPR014743	Chloride channel, core	Homologous Superfamily
A, B, C, D, E A, B, C, D, E, F	IPR000644	CBS domain	Domain
A, B, C, D, E A, B, C, D, E, F	IPR001807	Chloride channel, voltage gated	Family
A, B, C, D, E A, B, C, D, E, F	IPR002247	Chloride channel ClC-5	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B, C, D, E A, B, C, D, E, F	Pharos: P51795	Dent disease nephrolithiasis, X-linked recessive, with renal failure proteinuria, low molecular weight, with hypercalciuria and nephrocalcinosis hypophosphatemic rickets, X-linked recessive malignant glioma mixed glioma proteinuria X-linked dominant hypophosphatemic rickets goiter Dent disease type 1 osteosarcoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia acquired metabolic disease Sjogren syndrome Familial Hypophosphatemic Rickets Dent disease nephrolithiasis, X-linked recessive, with renal failure proteinuria, low molecular weight, with hypercalciuria and nephrocalcinosis hypophosphatemic rickets, X-linked recessive malignant glioma mixed glioma proteinuria X-linked dominant hypophosphatemic rickets goiter Dent disease type 1 osteosarcoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia acquired metabolic disease Sjogren syndrome Familial Hypophosphatemic Rickets glioma large cell medulloblastoma malignant mesothelioma nephrocalcinosis oculocerebrorenal syndrome ovarian cancer pituitary cancer adrenal cortex carcinoma tuberculosis X-linked hypophosphatemic rickets Fanconi renotubular syndrome medulloblastoma nephrolithiasis osteogenesis imperfecta pancreatic intraductal papillary-mucinous carcinoma primitive neuroectodermal tumor rheumatoid arthritis atypical teratoid rhabdoid tumor glioblastoma hypophosphatemia intellectual disability

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.