2PZF

Minimal human CFTR first nucleotide binding domain as a head-to-tail dimer with delta F508

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	d2pzfa1	Alpha and beta proteins (a/b)	P-loop containing nucleoside triphosphate hydrolases	P-loop containing nucleoside triphosphate hydrolases	ABC transporter ATPase domain-like	Cystic fibrosis transmembrane conductance regulator, CFTR, nucleotide-binding domain	Human (Homo sapiens ) [TaxId: 9606 ], Human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
A	d2pzfa2	Artifacts	Tags	Tags	Tags	N-terminal Tags	Human (Homo sapiens ) [TaxId: 9606 ], Human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d2pzfb_	Alpha and beta proteins (a/b)	P-loop containing nucleoside triphosphate hydrolases	P-loop containing nucleoside triphosphate hydrolases	ABC transporter ATPase domain-like	Cystic fibrosis transmembrane conductance regulator, CFTR, nucleotide-binding domain	Human (Homo sapiens ) [TaxId: 9606 ], 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	ABC transporter-like P-loop ATPases	8056723	3002020	SCOP2B (2022-06-29)
B	SCOP2B Superfamily	ABC transporter-like P-loop ATPases	8056723	3002020	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	ABC_tran_1	e2pzfA1	A: a/b three-layered sandwiches	X: P-loop domains-like	H: P-loop domains-related	T: P-loop containing nucleoside triphosphate hydrolases	F: ABC_tran_1	ECOD (1.6)
B	ABC_tran_1	e2pzfB1	A: a/b three-layered sandwiches	X: P-loop domains-like	H: P-loop domains-related	T: P-loop containing nucleoside triphosphate hydrolases	F: ABC_tran_1	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.40.50.300	Alpha Beta	3-Layer(aba) Sandwich	Rossmann fold	P-loop containing nucleotide triphosphate hydrolases	CATH (4.3.0)
B	3.40.50.300	Alpha Beta	3-Layer(aba) Sandwich	Rossmann fold	P-loop containing nucleotide triphosphate hydrolases	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B	PF00005	ABC transporter (ABC_tran)	ABC transporter	ABC transporters for a large family of proteins responsible for translocation of a variety of compounds across biological membranes. ABC transporters are the largest family of proteins in many completely sequenced bacteria. ABC transporters are comp ...	ABC transporters for a large family of proteins responsible for translocation of a variety of compounds across biological membranes. ABC transporters are the largest family of proteins in many completely sequenced bacteria. ABC transporters are composed of two copies of this domain and two copies of a transmembrane domain Pfam:PF00664. These four domains may belong to a single polypeptide as in Swiss:P13569, or belong in different polypeptide chains.	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B	Cystic fibrosis transmembrane conductance regulator	monoatomic anion transmembrane transporter activity chloride transmembrane transporter activity intracellularly ligand-gated monoatomic ion channel activity gated channel activity monoatomic ion channel activity intracellularly ATP-gated chloride channel activity intracellularly ATP-gated ion channel activity ligand-gated monoatomic anion channel activity ATP-gated ion channel activity ligand-gated channel activity ligand-gated monoatomic ion channel activity passive transmembrane transporter activity inorganic molecular entity transmembrane transporter activity channel activity monoatomic ion transmembrane transporter activity monoatomic anion transmembrane transporter activity chloride transmembrane transporter activity intracellularly ligand-gated monoatomic ion channel activity gated channel activity monoatomic ion channel activity intracellularly ATP-gated chloride channel activity intracellularly ATP-gated ion channel activity ligand-gated monoatomic anion channel activity ATP-gated ion channel activity ligand-gated channel activity ligand-gated monoatomic ion channel activity passive transmembrane transporter activity inorganic molecular entity transmembrane transporter activity channel activity monoatomic ion transmembrane transporter activity monoatomic anion channel activity transporter activity transmembrane transporter activity inorganic anion transmembrane transporter activity chloride channel activity ABC-type transporter activity active transmembrane transporter activity primary active transmembrane transporter activity ATPase-coupled transmembrane transporter activity ATP-dependent activity bicarbonate transmembrane transporter activity organic anion transmembrane transporter activity molecular function inhibitor activity chloride channel inhibitor activity channel regulator activity transporter inhibitor activity ion channel regulator activity ion channel inhibitor activity molecular function regulator activity transporter regulator activity channel inhibitor activity chloride channel regulator activity protein binding binding protein-folding chaperone binding 14-3-3 protein 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 Sec61 translocon complex binding protein-containing complex binding PDZ domain binding protein domain specific binding ATPase-coupled inorganic anion transmembrane transporter activity isomerase activity catalytic activity pyrophosphatase activity ATP hydrolysis activity ribonucleoside triphosphate phosphatase activity hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides hydrolase activity hydrolase activity, acting on acid anhydrides enzyme binding	developmental process spermatid development spermatid differentiation sexual reproduction cellular developmental process spermatogenesis reproductive process multicellular organismal reproductive process cell development cellular process involved in reproduction in multicellular organism developmental maturation developmental process involved in reproduction gamete generation male gamete generation sperm capacitation developmental process spermatid development spermatid differentiation sexual reproduction cellular developmental process spermatogenesis reproductive process multicellular organismal reproductive process cell development cellular process involved in reproduction in multicellular organism developmental maturation developmental process involved in reproduction gamete generation male gamete generation sperm capacitation anatomical structure maturation cell differentiation anatomical structure development germ cell development cellular process cell maturation transport monoatomic ion transmembrane transport inorganic ion transmembrane transport inorganic anion transmembrane transport establishment of localization chloride transport transmembrane transport localization chloride transmembrane transport monoatomic anion transport monoatomic anion transmembrane transport monoatomic ion transport inorganic anion transport macromolecule localization lipid localization cholesterol transport lipid transport sterol transport organic hydroxy compound transport bicarbonate transport organic anion transport regulation of localization regulation of cellular process positive regulation of biological process regulation of voltage-gated chloride channel activity regulation of transmembrane transporter activity regulation of monoatomic ion transmembrane transport regulation of transporter activity positive regulation of molecular function regulation of monoatomic anion transport positive regulation of monoatomic ion transport positive regulation of transmembrane transport positive regulation of monoatomic ion transmembrane transport positive regulation of cellular process regulation of chloride transport regulation of monoatomic ion transmembrane transporter activity regulation of biological process positive regulation of voltage-gated chloride channel activity regulation of transport positive regulation of anion channel activity regulation of monoatomic ion transport biological regulation regulation of monoatomic anion transmembrane transport regulation of anion channel activity positive regulation of anion transmembrane transport positive regulation of monoatomic anion transport regulation of molecular function regulation of transmembrane transport positive regulation of transport positive regulation of transporter activity positive regulation of ion transmembrane transporter activity positive regulation of inorganic anion transmembrane transport regulation of inorganic anion transmembrane transport transepithelial water transport epithelial fluid transport transepithelial transport fluid transport water transport anatomical structure morphogenesis odontogenesis of dentin-containing tooth anatomical structure formation involved in morphogenesis odontogenesis animal organ development amelogenesis animal organ morphogenesis regulation of biological quality membrane hyperpolarization regulation of membrane potential cellular response to stimulus cellular response to stress response to stimulus response to stress response to endoplasmic reticulum stress positive regulation of cyclic nucleotide-gated ion channel activity regulation of cyclic nucleotide-gated ion channel activity positive regulation of secretion by cell positive regulation of exocytosis vesicle-mediated transport regulation of secretion by cell secretion by cell positive regulation of secretion secretion export from cell regulation of vesicle-mediated transport regulation of secretion exocytosis regulation of exocytosis membrane docking organelle localization by membrane tethering organelle localization vesicle docking exocytic process vesicle docking involved in exocytosis regulation of body fluid levels multicellular organismal process multicellular organismal-level homeostasis chemical homeostasis homeostatic process multicellular organismal-level water homeostasis multicellular organismal-level chemical homeostasis enamel mineralization tooth mineralization regulation of developmental process regulation of biomineral tissue development positive regulation of enamel mineralization positive regulation of developmental process multicellular organism development positive regulation of biomineral tissue development biomineral tissue development regulation of multicellular organismal process regulation of multicellular organismal development tissue development regulation of tooth mineralization regulation of enamel mineralization positive regulation of tooth mineralization cellular response to nitrogen compound response to organophosphorus cellular response to organic cyclic compound response to oxygen-containing compound response to nitrogen compound response to purine-containing compound response to cAMP cellular response to oxygen-containing compound cellular response to chemical stimulus response to chemical cellular response to cAMP response to organic cyclic compound secondary alcohol biosynthetic process small molecule biosynthetic process cholesterol biosynthetic process organic hydroxy compound metabolic process sterol metabolic process steroid metabolic process cholesterol metabolic process lipid biosynthetic process alcohol metabolic process primary metabolic process secondary alcohol metabolic process biosynthetic process sterol biosynthetic process organic hydroxy compound biosynthetic process alcohol biosynthetic process small molecule metabolic process metabolic process steroid biosynthetic process lipid metabolic process cellular localization establishment of localization in cell protein localization positive regulation of cell communication establishment of protein localization nitrogen compound transport positive regulation of establishment of protein localization cell communication carbohydrate homeostasis cellular macromolecule localization positive regulation of insulin secretion involved in cellular response to glucose stimulus positive regulation of insulin secretion regulation of peptide hormone secretion regulation of hormone levels positive regulation of peptide hormone secretion establishment of protein localization to extracellular region intracellular glucose homeostasis protein secretion regulation of protein secretion cellular response to carbohydrate stimulus response to glucose glucose homeostasis positive regulation of protein localization insulin secretion involved in cellular response to glucose stimulus regulation of hormone secretion regulation of protein localization regulation of peptide secretion signaling hormone secretion protein localization to extracellular region positive regulation of protein transport peptide secretion positive regulation of hormone secretion response to monosaccharide response to carbohydrate amide transport signal release cellular response to hexose stimulus intracellular chemical homeostasis cellular response to monosaccharide stimulus positive regulation of peptide secretion regulation of signaling regulation of peptide transport regulation of establishment of protein localization cell-cell signaling cellular response to glucose stimulus regulation of protein transport positive regulation of protein secretion peptide transport response to hexose cellular homeostasis protein transport regulation of cellular localization regulation of insulin secretion peptide hormone secretion regulation of insulin secretion involved in cellular response to glucose stimulus hormone transport regulation of cell communication insulin secretion positive regulation of signaling regulation of intracellular pH intracellular monoatomic cation homeostasis regulation of pH monoatomic ion homeostasis monoatomic cation homeostasis intracellular pH elevation regulation of cellular pH intracellular monoatomic ion homeostasis response to ketone response to lipid response to alcohol cellular response to forskolin cellular response to alcohol response to forskolin cellular response to lipid cellular response to ketone	protein-containing complex cellular anatomical structure transmembrane transporter complex membrane chloride channel complex membrane protein complex transporter complex monoatomic ion channel complex membrane-bounded organelle intracellular anatomical structure intracellular vesicle intracellular organelle bounding membrane of organelle cytoplasmic vesicle protein-containing complex cellular anatomical structure transmembrane transporter complex membrane chloride channel complex membrane protein complex transporter complex monoatomic ion channel complex membrane-bounded organelle intracellular anatomical structure intracellular vesicle intracellular organelle bounding membrane of organelle cytoplasmic vesicle vesicle membrane clathrin-coated vesicle endocytic vesicle membrane coated vesicle vesicle endocytic vesicle clathrin-coated vesicle membrane clathrin-coated endocytic vesicle membrane intracellular membrane-bounded organelle clathrin-coated endocytic vesicle cytoplasmic vesicle membrane coated vesicle membrane organelle cytoplasm organelle membrane cytosol vacuolar membrane lytic vacuole vacuole lytic vacuole membrane lysosomal membrane lysosome endosome recycling endosome endomembrane system endosome membrane Golgi-associated vesicle membrane Golgi-associated vesicle cell periphery apical part of cell plasma membrane plasma membrane region apical plasma membrane cell surface organelle subcompartment endoplasmic reticulum subcompartment nuclear outer membrane-endoplasmic reticulum membrane network endoplasmic reticulum endoplasmic reticulum membrane early endosome recycling endosome membrane early endosome membrane nucleus

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B	IPR047082	Cystic fibrosis transmembrane conductance regulator, ATP-binding cassette domain 1	Domain
A, B	IPR003439	ABC transporter-like, ATP-binding domain	Domain
A, B	IPR009147	Cystic fibrosis transmembrane conductance regulator	Family
A, B	IPR025837	CFTR regulator domain	Domain
A, B	IPR027417	P-loop containing nucleoside triphosphate hydrolase	Homologous Superfamily
A, B	IPR003593	AAA+ ATPase domain	Domain
A, B	IPR011527	ABC transporter type 1, transmembrane domain	Domain
A, B	IPR017871	ABC transporter-like, conserved site	Conserved Site
A, B	IPR036640	ABC transporter type 1, transmembrane domain superfamily	Homologous Superfamily
A, B	IPR050173	ATP-binding cassette transporter C-like	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B	Pharos: P13569	cystic fibrosis pancreatitis rhinitis bronchiectasis with or without elevated sweat chloride 1 autosomal recessive polycystic kidney disease Congenital bilateral aplasia of vas deferens sinusitis esophageal adenocarcinoma azoospermia male infertility chronic pancreatitis congenital bilateral aplasia of vas deferens from CFTR mutation hereditary chronic pancreatitis choledochal cyst colorectal cancer cystic fibrosis pancreatitis rhinitis bronchiectasis with or without elevated sweat chloride 1 autosomal recessive polycystic kidney disease Congenital bilateral aplasia of vas deferens sinusitis esophageal adenocarcinoma azoospermia male infertility chronic pancreatitis congenital bilateral aplasia of vas deferens from CFTR mutation hereditary chronic pancreatitis choledochal cyst colorectal cancer pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia congenital diaphragmatic hernia drug dependence endogenous depression heart disease substance dependence bronchiectasis lung adenocarcinoma lung disease colorectal carcinoma pancreatic intraductal papillary-mucinous carcinoma cutaneous lupus erythematosus psoriasis substance abuse attention deficit hyperactivity disorder, inattentive type hemochromatosis type 1 idiopathic bronchiectasis Tourette syndrome lung cancer malignant mesothelioma non-small cell lung carcinoma pancreatic intraductal papillary-mucinous neoplasm plantar fibromatosis Sjogren syndrome substance-related disorder Barrett esophagus inflammatory bowel disease ulcerative colitis lung carcinoma male infertility with azoospermia or oligozoospermia due to single gene mutation cholestasis malignant pancreatic neoplasm

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.