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Protein

Cystic fibrosis transmembrane conductance regulator

Gene

cftr

Organism
Danio rerio (Zebrafish) (Brachydanio rerio)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis (PubMed:20933420, PubMed:23487313, PubMed:25592226). Mediates the transport of chloride ions across the cell membrane (By similarity). Channel activity is coupled to ATP hydrolysis. The ion channel is also permeable to HCO3-; selectivity depends on the extracellular chloride concentration. Exerts its function also by modulating the activity of other ion channels and transporters. Contributes to the regulation of the pH and the ion content of the epithelial fluid layer (By similarity). Required for normal fluid homeostasis in the gut (PubMed:20933420). Required for normal volume expansion of Kupffer's vesicle during embryonic development and for normal establishment of left-right body patterning (PubMed:23487313, PubMed:26432887). Required for normal resistance to infection by P.aeruginosa strain PA14 and strain SMC573 (PubMed:20732993).By similarity1 Publication4 Publications

Miscellaneous

Mutations that lead to the production of a severely truncated protein that ends before the start of the fourth transmembrane domain disrupt normal left-right body patterning during embryogenesis and abolish lumen expansion of Kupffer's vesicle.1 Publication

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section describes biophysical and chemical properties, such as maximal absorption, kinetic parameters, pH dependence, redox potentials and temperature dependence.<p><a href='/help/biophysicochemical_properties' target='_top'>More...</a></p>Kineticsi

  1. KM=0.22 mM for ATP1 Publication

    Sites

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei402ATP 1By similarity1
    Binding sitei492ATP 1By similarity1
    Binding sitei1220ATP 2By similarity1

    Regions

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Function’ section describes a region in the protein which binds nucleotide phosphates. It always involves more than one amino acid and includes all residues involved in nucleotide-binding.<p><a href='/help/np_bind' target='_top'>More...</a></p>Nucleotide bindingi457 – 464ATP 1PROSITE-ProRule annotation8
    Nucleotide bindingi1245 – 1252ATP 2PROSITE-ProRule annotation8

    <p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

    GO - Biological processi

    <p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

    Molecular functionChloride channel, Hydrolase, Ion channel
    Biological processIon transport, Transport
    LigandATP-binding, Chloride, Nucleotide-binding

    Enzyme and pathway databases

    Reactome - a knowledgebase of biological pathways and processes

    More...
    Reactomei
    R-DRE-382556 ABC-family proteins mediated transport
    R-DRE-5627083 RHO GTPases regulate CFTR trafficking
    R-DRE-5689880 Ub-specific processing proteases
    R-DRE-8856825 Cargo recognition for clathrin-mediated endocytosis
    R-DRE-8856828 Clathrin-mediated endocytosis

    Protein family/group databases

    Transport Classification Database

    More...
    TCDBi
    3.A.1.202.2 the atp-binding cassette (abc) superfamily

    <p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
    Recommended name:
    Cystic fibrosis transmembrane conductance regulatorUniRule annotation (EC:3.6.3.49UniRule annotation1 Publication)
    Alternative name(s):
    ATP-binding cassette sub-family C member 7UniRule annotation
    Channel conductance-controlling ATPaseUniRule annotation
    cAMP-dependent chloride channelUniRule annotation
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
    Name:cftrImported
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiDanio rerio (Zebrafish) (Brachydanio rerio)Imported
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri7955 [NCBI]
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiActinopterygiiNeopterygiiTeleosteiOstariophysiCypriniformesCyprinidaeDanio
    <p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
    • UP000000437 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 18

    Organism-specific databases

    Zebrafish Information Network genome database

    More...
    ZFINi
    ZDB-GENE-050517-20 cftr

    <p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

    Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

    Topology

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/help/subcellular_location_section">'Subcellular location'</a> section describes the subcellular compartment where each non-membrane region of a membrane-spanning protein is found.<p><a href='/help/topo_dom' target='_top'>More...</a></p>Topological domaini1 – 78Cytoplasmic1 PublicationAdd BLAST78
    <p>This subsection of the <a href="http://www.uniprot.org/help/subcellular_location_section">'Subcellular location'</a> section describes the extent of a membrane-spanning region of the protein. It denotes the presence of both alpha-helical transmembrane regions and the membrane spanning regions of beta-barrel transmembrane proteins.<p><a href='/help/transmem' target='_top'>More...</a></p>Transmembranei79 – 99Helical; Name=11 PublicationAdd BLAST21
    Topological domaini100 – 123Extracellular1 PublicationAdd BLAST24
    Transmembranei124 – 149Helical; Name=21 PublicationAdd BLAST26
    Topological domaini150 – 195Cytoplasmic1 PublicationAdd BLAST46
    Transmembranei196 – 216Helical; Name=31 PublicationAdd BLAST21
    Topological domaini217 – 224Extracellular1 Publication8
    Transmembranei225 – 245Helical; Name=41 PublicationAdd BLAST21
    Topological domaini246 – 299Cytoplasmic1 PublicationAdd BLAST54
    Transmembranei300 – 320Helical; Name=51 PublicationAdd BLAST21
    Topological domaini321 – 340Extracellular1 PublicationAdd BLAST20
    Transmembranei341 – 363Helical; Name=61 PublicationAdd BLAST23
    Topological domaini364 – 856Cytoplasmic1 PublicationAdd BLAST493
    Transmembranei857 – 877Helical; Name=71 PublicationAdd BLAST21
    Topological domaini878 – 924Extracellular1 PublicationAdd BLAST47
    Transmembranei925 – 946Discontinuously helical; Name=81 PublicationAdd BLAST22
    Topological domaini947 – 996Cytoplasmic1 PublicationAdd BLAST50
    Transmembranei997 – 1019Helical; Name=91 PublicationAdd BLAST23
    Topological domaini1020 – 1021Extracellular1 Publication2
    Transmembranei1022 – 1042Helical; Name=101 PublicationAdd BLAST21
    Topological domaini1043 – 1103Cytoplasmic1 PublicationAdd BLAST61
    Transmembranei1104 – 1124Helical; Name=111 PublicationAdd BLAST21
    Topological domaini1125 – 1138Extracellular1 PublicationAdd BLAST14
    Transmembranei1139 – 1159Helical; Name=121 PublicationAdd BLAST21
    Topological domaini1160 – 1485Cytoplasmic1 PublicationAdd BLAST326

    Keywords - Cellular componenti

    Cell membrane, Endoplasmic reticulum, Endosome, Membrane

    <p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

    <p>This subsection of the ‘Pathology and Biotech’ section describes the in vivo effects caused by ablation of the gene (or one or more transcripts) coding for the protein described in the entry. This includes gene knockout and knockdown, provided experiments have been performed in the context of a whole organism or a specific tissue, and not at the single-cell level.<p><a href='/help/disruption_phenotype' target='_top'>More...</a></p>Disruption phenotypei

    Considerable lethality around 10 dpf (PubMed:25592226). No effect on initial pancreas development, but at 16 dpf mutants display loss of pancreatic acinar tissue (PubMed:25592226). At 22 dpf, most pancreatic acinar tissue has disappeared and has been replaced by fibrotic tissue that surrounds dilated, mucus-filled ducts (PubMed:25592226). Morpholino knockdown of the protein in 48 hpf embryos leads to impaired resistance to P.aeruginosa strain PA14 and strain SMC573, as shown by the increased bacterial burden, but there is no effect on resistance to E.tarda, B.cenocepacia, S.aureus MZ100, E.coli XL-10 and H.influenzae Hib EAGAN (PubMed:20732993). Morpholino knockdown of the protein causes an importanr reduction of the volume of Kupffer's vesicle during embryonic development (PubMed:26432887).3 Publications

    Mutagenesis

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi205 – 206Missing in pd1048; abolishes trafficking to the cell membrane and leads to severe reduction of the size of the Kupffer's vesicle. 1 Publication2

    <p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00004396701 – 1485Cystic fibrosis transmembrane conductance regulatorAdd BLAST1485

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM / Processing</a> section specifies the position and type of each covalently attached glycan group (mono-, di-, or polysaccharide).<p><a href='/help/carbohyd' target='_top'>More...</a></p>Glycosylationi897N-linked (GlcNAc...) asparaginePROSITE-ProRule annotation1
    Glycosylationi903N-linked (GlcNAc...) asparaginePROSITE-ProRule annotation1

    <p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

    Phosphorylated; this activates the channel (PubMed:27912062). Dephosphorylation strongly decreases ATPase activity (PubMed:27912062). Phosphorylation at PKA sites activates the channel. Phosphorylation at PKC sites enhances the response to phosphorylation by PKA (By similarity).By similarity1 Publication

    Keywords - PTMi

    Glycoprotein, Phosphoprotein

    Proteomic databases

    PaxDb, a database of protein abundance averages across all three domains of life

    More...
    PaxDbi
    Q1LX78

    <p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

    <p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

    Detected in gut epithelium (at protein level) (PubMed:20933420). Detected in kidney, spleen, intestine and liver (PubMed:20732993). Detected in pancreatic duct epithelium at 5 dpf and throughout adult life (PubMed:25592226).2 Publications

    <p>This subsection of the ‘Expression’ section provides information on the expression of the gene product at various stages of a cell, tissue or organism development. By default, the information is derived from experiments at the mRNA level, unless specified ‘at the protein level’.<p><a href='/help/developmental_stage' target='_top'>More...</a></p>Developmental stagei

    First detected in developing pancreatic duct at 3 dpf (PubMed:25592226). Detected on Kupffer's vesicle during embryonic development (PubMed:23487313, PubMed:26432887). Detected on neural floorplate, brain and pronephric duct primordia in embryos at the 10 somite stage (PubMed:26432887).3 Publications

    Gene expression databases

    Bgee dataBase for Gene Expression Evolution

    More...
    Bgeei
    ENSDARG00000041107 Expressed in 15 organ(s), highest expression level in testis

    <p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

    <p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

    Monomer; does not require oligomerization for channel activity (By similarity). Interacts with cse1l; this interaction may downregulate cftr activity (PubMed:20933420).By similarity1 Publication

    Protein-protein interaction databases

    STRING: functional protein association networks

    More...
    STRINGi
    7955.ENSDARP00000060242

    <p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

    Secondary structure

    11485
    Legend: HelixTurnBeta strandPDB Structure known for this area
    Show more details

    3D structure databases

    Select the link destinations:

    Protein Data Bank Europe

    More...
    PDBei

    Protein Data Bank RCSB

    More...
    RCSB PDBi

    Protein Data Bank Japan

    More...
    PDBji
    Links Updated
    PDB entryMethodResolution (Å)ChainPositionsPDBsum
    5UARelectron microscopy3.73A1-1485[»]
    5W81electron microscopy3.37A1-1485[»]

    Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

    More...
    ProteinModelPortali
    Q1LX78

    SWISS-MODEL Repository - a database of annotated 3D protein structure models

    More...
    SMRi
    Q1LX78

    Database of comparative protein structure models

    More...
    ModBasei
    Search...

    MobiDB: a database of protein disorder and mobility annotations

    More...
    MobiDBi
    Search...

    <p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/help/family_and_domains_section">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini83 – 353ABC transmembrane type-1 1PROSITE-ProRule annotationAdd BLAST271
    Domaini424 – 645ABC transporter 1PROSITE-ProRule annotationAdd BLAST222
    Domaini860 – 1163ABC transmembrane type-1 2PROSITE-ProRule annotationAdd BLAST304
    Domaini1211 – 1444ABC transporter 2PROSITE-ProRule annotationAdd BLAST234

    Region

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni653 – 826Intrinsically disordered R regionBy similarityAdd BLAST174

    Motif

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Family and Domains’ section describes a short (usually not more than 20 amino acids) conserved sequence motif of biological significance.<p><a href='/help/motif' target='_top'>More...</a></p>Motifi1483 – 1485PDZ-bindingBy similarity3

    <p>This subsection of the ‘Family and domains’ section provides general information on the biological role of a domain. The term ‘domain’ is intended here in its wide acceptation, it may be a structural domain, a transmembrane region or a functional domain. Several domains are described in this subsection.<p><a href='/help/domain_cc' target='_top'>More...</a></p>Domaini

    Binds and hydrolyzes ATP via the two cytoplasmic ABC transporter nucleotide-binding domains. The two ATP-binding domains interact with each other, forming a head-to-tail dimer. Normal ATPase activity requires interaction between the two domains. The first ABC transporter nucleotide-binding domain has no ATPase activity by itself.By similarity
    The R region is intrinsically disordered. It mediates channel activation when it is phosphorylated, but not in the absence of phosphorylation.By similarity

    <p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

    Belongs to the ABC transporter superfamily. ABCC family. CFTR transporter (TC 3.A.1.202) subfamily. [View classification]UniRule annotation

    Keywords - Domaini

    Repeat, Transmembrane, Transmembrane helix

    Phylogenomic databases

    evolutionary genealogy of genes: Non-supervised Orthologous Groups

    More...
    eggNOGi
    KOG0054 Eukaryota
    COG1132 LUCA

    Ensembl GeneTree

    More...
    GeneTreei
    ENSGT00940000158567

    The HOVERGEN Database of Homologous Vertebrate Genes

    More...
    HOVERGENi
    HBG004169

    InParanoid: Eukaryotic Ortholog Groups

    More...
    InParanoidi
    Q1LX78

    KEGG Orthology (KO)

    More...
    KOi
    K05031

    Identification of Orthologs from Complete Genome Data

    More...
    OMAi
    RCFFWKF

    Database of Orthologous Groups

    More...
    OrthoDBi
    138195at2759

    Database for complete collections of gene phylogenies

    More...
    PhylomeDBi
    Q1LX78

    TreeFam database of animal gene trees

    More...
    TreeFami
    TF105200

    Family and domain databases

    Gene3D Structural and Functional Annotation of Protein Families

    More...
    Gene3Di
    1.20.1560.10, 2 hits

    Integrated resource of protein families, domains and functional sites

    More...
    InterProi
    View protein in InterPro
    IPR003593 AAA+_ATPase
    IPR011527 ABC1_TM_dom
    IPR036640 ABC1_TM_sf
    IPR003439 ABC_transporter-like
    IPR017871 ABC_transporter_CS
    IPR009147 CFTR/ABCC7
    IPR025837 CFTR_reg_dom
    IPR027417 P-loop_NTPase

    The PANTHER Classification System

    More...
    PANTHERi
    PTHR24223:SF19 PTHR24223:SF19, 1 hit

    Pfam protein domain database

    More...
    Pfami
    View protein in Pfam
    PF00664 ABC_membrane, 2 hits
    PF00005 ABC_tran, 2 hits
    PF14396 CFTR_R, 1 hit

    Protein Motif fingerprint database; a protein domain database

    More...
    PRINTSi
    PR01851 CYSFIBREGLTR

    Simple Modular Architecture Research Tool; a protein domain database

    More...
    SMARTi
    View protein in SMART
    SM00382 AAA, 2 hits

    Superfamily database of structural and functional annotation

    More...
    SUPFAMi
    SSF52540 SSF52540, 2 hits
    SSF90123 SSF90123, 2 hits

    TIGRFAMs; a protein family database

    More...
    TIGRFAMsi
    TIGR01271 CFTR_protein, 1 hit

    PROSITE; a protein domain and family database

    More...
    PROSITEi
    View protein in PROSITE
    PS50929 ABC_TM1F, 2 hits
    PS00211 ABC_TRANSPORTER_1, 1 hit
    PS50893 ABC_TRANSPORTER_2, 2 hits

    <p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

    <p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

    This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

    Q1LX78-1 [UniParc]FASTAAdd to basket
    « Hide
            10         20         30         40         50
    MQRSPVEDAN CLSRYFFWWT NPIMRKGFKE KLRPSDVYQA PSQDAADILA
    60 70 80 90 100
    ERLEKEWDRE VASGKKKPSL LRAMARCYIK PFLLFGFLLY IGEATKTVQP
    110 120 130 140 150
    QLLGRIIASF DPAHEPERAN GYFLAFGLGL LFTARFLLLQ PAMFGLHHLG
    160 170 180 190 200
    MQIRIALFSI IYKKTLKLSS RVLDKISTGQ LVSLMSANLG KFDQSLGMAH
    210 220 230 240 250
    FIWISPLQCI LCTGLIWELI DVNSFCALAA ISLLGVLQAF LSHKMGPYKA
    260 270 280 290 300
    QKVLLTNKRL ALTSEIMENL HSVKAYGWEE IMETLIKNIR QDEVKLTRKI
    310 320 330 340 350
    GSLRYFYSSA YFFSAIFVIV AAVVPHALSR GINLRRIFTT LSYCMVLRMT
    360 370 380 390 400
    VTRQLPGSIQ MWYDTMRLIW KIEEFLSKEE YKLMEYDLSI TELELQDVTA
    410 420 430 440 450
    SWDEGPGELL ERIKQENKAN GHHNGDAGLF FTNLYVAPVL KDISLKLKKG
    460 470 480 490 500
    EMLAVTGSMG SGKSSLLMTI LGELVPSSGK IRHSGRISYS SQTAWIMPGT
    510 520 530 540 550
    IRDNILFGLT YDEYRYKSVV KACQLEEDLA ALPEKDKTPM AEGGLNLSGG
    560 570 580 590 600
    QKARVALARA VYRDADLYLL DAPFTHLDIA TEKEIFDKCL CKLMASKTRI
    610 620 630 640 650
    LVTNKIEHLK RADKILLLHN GESFFYGTFP ELQSERPDFS SLLLGLEAYD
    660 670 680 690 700
    NISAERRCSI LTETLHRVSV DESAGMQPER SAFRQVPPTK PMYIDERKAS
    710 720 730 740 750
    VIVNPLGVAR KASFIQVPEE EVRRTLPDRK FSLVPENELV DESFMGSDVY
    760 770 780 790 800
    HNHGVHMAGQ RRQSVLAFMT NAQGQGRREH LQSSFRRRLS VVPQSELASE
    810 820 830 840 850
    LDIYTRRLSD STYDMTGILE EENIEACLTD EIDEIEETFE TTKWNTYVRY
    860 870 880 890 900
    VSNNKSLLYV LIFILFIAAI EIAGSVAGIF LITDELWREE HQRSEPNMTK
    910 920 930 940 950
    HSNASSSGQT YAITVTPTSS YYILYIYVAT SESLLAMGFF RGLPFVHTTI
    960 970 980 990 1000
    TISKKLHQKM LHAVLSAPMS VLNTMKTGRI MNRFTKDMAT IDDMLPLLMF
    1010 1020 1030 1040 1050
    DFVQLTVVVV GCILVVSIVR PYIFLAATPL AIIFIVMRKY FLRTGQQLKQ
    1060 1070 1080 1090 1100
    LETEARSPIF SHLIMSLKGL WTIRAFERQA YFEALFHKTL NTHTATWFLY
    1110 1120 1130 1140 1150
    LSTLRWFLFR ADILFVFFFT LAAWIAVGTN QDKPGEIGII ICLAMLILGT
    1160 1170 1180 1190 1200
    FQWCVATSIA VDGMMRSVDR VFKFIDLPSE TPKPDKGKDS DLIIENVDAQ
    1210 1220 1230 1240 1250
    ADSSWPHRGQ IEVRNLTVKY TEAGHAVLKN LSFSAEGRQR VGILGRTGSG
    1260 1270 1280 1290 1300
    KSSLFNALLK LVYTDGEISI DGVNWNKMPL QKWRKAFGVV PQKVFIFTGP
    1310 1320 1330 1340 1350
    LRMNLDPYGC HSDEELWRVA EEVGLKTVIE QFPDKLDFQL EYGGYVLSNG
    1360 1370 1380 1390 1400
    HKQLICLARS ILSGARILLL DEPSAHLDPV TIKVLKKTLR QSFSTCTILL
    1410 1420 1430 1440 1450
    SEHKVEPLLE CQSFLMMDKG QVKTYDSIQK LLNETSHLKQ AISPAERLKL
    1460 1470 1480
    FPRRNSSMRT PQSKLSSVTQ TLQEEAEDNI QDTRL
    Length:1,485
    Mass (Da):168,400
    Last modified:May 30, 2006 - v1
    <p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i62601EBC307A3B8A
    GO

    <p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

    There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
    EntryEntry nameProtein names
    Gene namesLengthAnnotation
    A0A0R4ID63A0A0R4ID63_DANRE
    Cystic fibrosis transmembrane condu...
    cftr
    1,485Annotation score:

    Annotation score:3 out of 5

    <p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti658C → S in AAI71654 (Ref. 2) Curated1

    Sequence databases

    Select the link destinations:

    EMBL nucleotide sequence database

    More...
    EMBLi

    GenBank nucleotide sequence database

    More...
    GenBanki

    DNA Data Bank of Japan; a nucleotide sequence database

    More...
    DDBJi
    Links Updated
    BX470130 Genomic DNA No translation available.
    BC171654 mRNA Translation: AAI71654.1

    NCBI Reference Sequences

    More...
    RefSeqi
    NP_001038348.1, NM_001044883.1

    UniGene gene-oriented nucleotide sequence clusters

    More...
    UniGenei
    Dr.74099

    Genome annotation databases

    Ensembl eukaryotic genome annotation project

    More...
    Ensembli
    ENSDART00000060243; ENSDARP00000060242; ENSDARG00000041107

    Database of genes from NCBI RefSeq genomes

    More...
    GeneIDi
    559080

    KEGG: Kyoto Encyclopedia of Genes and Genomes

    More...
    KEGGi
    dre:559080

    <p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

    <p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    BX470130 Genomic DNA No translation available.
    BC171654 mRNA Translation: AAI71654.1
    RefSeqiNP_001038348.1, NM_001044883.1
    UniGeneiDr.74099

    3D structure databases

    Select the link destinations:
    PDBei
    RCSB PDBi
    PDBji
    Links Updated
    PDB entryMethodResolution (Å)ChainPositionsPDBsum
    5UARelectron microscopy3.73A1-1485[»]
    5W81electron microscopy3.37A1-1485[»]
    ProteinModelPortaliQ1LX78
    SMRiQ1LX78
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    STRINGi7955.ENSDARP00000060242

    Protein family/group databases

    TCDBi3.A.1.202.2 the atp-binding cassette (abc) superfamily

    Proteomic databases

    PaxDbiQ1LX78

    Protocols and materials databases

    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

    EnsembliENSDART00000060243; ENSDARP00000060242; ENSDARG00000041107
    GeneIDi559080
    KEGGidre:559080

    Organism-specific databases

    Comparative Toxicogenomics Database

    More...
    CTDi
    1080
    ZFINiZDB-GENE-050517-20 cftr

    Phylogenomic databases

    eggNOGiKOG0054 Eukaryota
    COG1132 LUCA
    GeneTreeiENSGT00940000158567
    HOVERGENiHBG004169
    InParanoidiQ1LX78
    KOiK05031
    OMAiRCFFWKF
    OrthoDBi138195at2759
    PhylomeDBiQ1LX78
    TreeFamiTF105200

    Enzyme and pathway databases

    ReactomeiR-DRE-382556 ABC-family proteins mediated transport
    R-DRE-5627083 RHO GTPases regulate CFTR trafficking
    R-DRE-5689880 Ub-specific processing proteases
    R-DRE-8856825 Cargo recognition for clathrin-mediated endocytosis
    R-DRE-8856828 Clathrin-mediated endocytosis

    Miscellaneous databases

    Protein Ontology

    More...
    PROi
    PR:Q1LX78

    Gene expression databases

    BgeeiENSDARG00000041107 Expressed in 15 organ(s), highest expression level in testis

    Family and domain databases

    Gene3Di1.20.1560.10, 2 hits
    InterProiView protein in InterPro
    IPR003593 AAA+_ATPase
    IPR011527 ABC1_TM_dom
    IPR036640 ABC1_TM_sf
    IPR003439 ABC_transporter-like
    IPR017871 ABC_transporter_CS
    IPR009147 CFTR/ABCC7
    IPR025837 CFTR_reg_dom
    IPR027417 P-loop_NTPase
    PANTHERiPTHR24223:SF19 PTHR24223:SF19, 1 hit
    PfamiView protein in Pfam
    PF00664 ABC_membrane, 2 hits
    PF00005 ABC_tran, 2 hits
    PF14396 CFTR_R, 1 hit
    PRINTSiPR01851 CYSFIBREGLTR
    SMARTiView protein in SMART
    SM00382 AAA, 2 hits
    SUPFAMiSSF52540 SSF52540, 2 hits
    SSF90123 SSF90123, 2 hits
    TIGRFAMsiTIGR01271 CFTR_protein, 1 hit
    PROSITEiView protein in PROSITE
    PS50929 ABC_TM1F, 2 hits
    PS00211 ABC_TRANSPORTER_1, 1 hit
    PS50893 ABC_TRANSPORTER_2, 2 hits

    ProtoNet; Automatic hierarchical classification of proteins

    More...
    ProtoNeti
    Search...

    <p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

    <p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiCFTR_DANRE
    <p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q1LX78
    Secondary accession number(s): B7ZVN9
    <p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 12, 2017
    Last sequence update: May 30, 2006
    Last modified: January 16, 2019
    This is version 113 of the entry and version 1 of the sequence. See complete history.
    <p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    <p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

    Keywords - Technical termi

    3D-structure, Complete proteome, Reference proteome

    Documents

    1. SIMILARITY comments
      Index of protein domains and families
    2. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
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