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Search results 901 to 1000 out of 1123 for Cftr

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Type Details Score
Publication      
First Author: Mouse Genome Informatics (MGI) and The National Center for Biotechnology Information (NCBI)
Year: 2010
Journal: Database Download
Title: Consensus CDS project
Publication      
First Author: Mouse Genome Informatics Scientific Curators
Year: 2009
Journal: Database Download
Title: Mouse Microarray Data Integration in Mouse Genome Informatics, the Affymetrix GeneChip Mouse Genome 430 2.0 Array Platform
Publication      
First Author: Mouse Genome Informatics Scientific Curators
Year: 2009
Journal: Database Download
Title: Mouse Microarray Data Integration in Mouse Genome Informatics, the Affymetrix GeneChip Mouse Gene 1.0 ST Array Platform
Publication      
First Author: Mouse Genome Informatics
Year: 2010
Journal: Database Release
Title: Protein Ontology Association Load.
Publication        
First Author: Mouse Genome Informatics Scientific Curators
Year: 2001
Title: Gene Ontology Annotation by the MGI Curatorial Staff
Publication      
First Author: The Jackson Laboratory Mouse Radiation Hybrid Database
Year: 2004
Journal: Database Release
Title: Mouse T31 Radiation Hybrid Data Load
Pathway
Publication
First Author: Shin YH
Year: 2016
Journal: Biochem Biophys Res Commun
Title: Epigenetic regulation of CFTR in salivary gland.
Volume: 481
Issue: 1-2
Pages: 31-37
HT Experiment
Series Id: GSE75996
Experiment Type: transcription profiling by array
Study Type: WT vs. Mutant
Source: ArrayExpress
Publication
First Author: Nakanishi K
Year: 2001
Journal: J Am Soc Nephrol
Title: Role of CFTR in autosomal recessive polycystic kidney disease.
Volume: 12
Issue: 4
Pages: 719-25
Publication
First Author: Yang B
Year: 2008
Journal: J Am Soc Nephrol
Title: Small-molecule CFTR inhibitors slow cyst growth in polycystic kidney disease.
Volume: 19
Issue: 7
Pages: 1300-10
GO Term
HT Experiment
Series Id: GSE5715
Experiment Type: transcription profiling by array
Study Type: WT vs. Mutant
Source: ArrayExpress
Publication
First Author: Wang YY
Year: 2017
Journal: PLoS Genet
Title: Loss of SLC9A3 decreases CFTR protein and causes obstructed azoospermia in mice.
Volume: 13
Issue: 4
Pages: e1006715
Publication
First Author: Manavalan P
Year: 1995
Journal: FEBS Lett
Title: Sequence homologies between nucleotide binding regions of CFTR and G-proteins suggest structural and functional similarities.
Volume: 366
Issue: 2-3
Pages: 87-91
Publication
First Author: Chow YH
Year: 2000
Journal: Mol Ther
Title: Targeting transgene expression to airway epithelia and submucosal glands, prominent sites of human CFTR expression.
Volume: 2
Issue: 4
Pages: 359-67
Publication
First Author: Rowntree RK
Year: 2001
Journal: Hum Mol Genet
Title: An element in intron 1 of the CFTR gene augments intestinal expression in vivo.
Volume: 10
Issue: 14
Pages: 1455-64
Publication
First Author: Hegedus T
Year: 2009
Journal: Biochim Biophys Acta
Title: Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A.
Volume: 1788
Issue: 6
Pages: 1341-9
Publication
First Author: Prieto S
Year: 2023
Journal: EMBO Rep
Title: CDK8 and CDK19 act redundantly to control the CFTR pathway in the intestinal epithelium.
Volume: 24
Issue: 2
Pages: e54261
Publication
First Author: Haque A
Year: 2010
Journal: Nucleic Acids Res
Title: Functional properties and evolutionary splicing constraints on a composite exonic regulatory element of splicing in CFTR exon 12.
Volume: 38
Issue: 2
Pages: 647-59
Interaction Experiment
Description: Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis.
Allele
Name: cystic fibrosis transmembrane conductance regulator; endonuclease-mediated mutation 1, Shanghai Model Organisms Center
Allele Type: Endonuclease-mediated
Attribute String: Null/knockout
Publication
First Author: Yanda MK
Year: 2018
Journal: J Biol Chem
Title: A potential strategy for reducing cysts in autosomal dominant polycystic kidney disease with a CFTR corrector.
Volume: 293
Issue: 29
Pages: 11513-11526
Publication
First Author: Shcheynikov N
Year: 2008
Journal: J Physiol
Title: The Slc26a4 transporter functions as an electroneutral Cl-/I-/HCO3- exchanger: role of Slc26a4 and Slc26a6 in I- and HCO3- secretion and in regulation of CFTR in the parotid duct.
Volume: 586
Issue: 16
Pages: 3813-24
Publication
First Author: Abdulrahman BA
Year: 2013
Journal: J Biol Chem
Title: Depletion of the ubiquitin-binding adaptor molecule SQSTM1/p62 from macrophages harboring cftr ΔF508 mutation improves the delivery of Burkholderia cenocepacia to the autophagic machinery.
Volume: 288
Issue: 3
Pages: 2049-58
Publication
First Author: Kim Chiaw P
Year: 2019
Journal: PLoS One
Title: Hsp70 and DNAJA2 limit CFTR levels through degradation.
Volume: 14
Issue: 8
Pages: e0220984
Allele
Name: cystic fibrosis transmembrane conductance regulator; targeted mutation 1, Kirk R Thomas
Allele Type: Targeted
Attribute String: Hypomorph
Publication
First Author: Dean M
Year: 2001
Journal: Genome Res
Title: The human ATP-binding cassette (ABC) transporter superfamily.
Volume: 11
Issue: 7
Pages: 1156-66
CL Term
Protein
Organism: Mus musculus/domesticus
Length: 463  
Fragment?: false
DO Term
GO Term
Publication
First Author: Thibodeau PH
Year: 2005
Journal: Nat Struct Mol Biol
Title: Side chain and backbone contributions of Phe508 to CFTR folding.
Volume: 12
Issue: 1
Pages: 10-6
Protein
Organism: Mus musculus/domesticus
Length: 1446  
Fragment?: false
Allele
Name: cystic fibrosis transmembrane conductance regulator; targeted mutation 1, University of North Carolina
Allele Type: Targeted
Attribute String: Null/knockout
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein
Organism: Mus musculus/domesticus
Length: 205  
Fragment?: false
Allele
Name: transgene insertion 1, Michael J Welsh
Allele Type: Transgenic
Attribute String: Humanized sequence, Inserted expressed sequence
Protein
Organism: Mus musculus/domesticus
Length: 600  
Fragment?: false
Protein
Organism: Mus musculus/domesticus
Length: 576  
Fragment?: false
Publication
First Author: Mendoza JL
Year: 2012
Journal: Cell
Title: Requirements for efficient correction of ΔF508 CFTR revealed by analyses of evolved sequences.
Volume: 148
Issue: 1-2
Pages: 164-74
Publication
First Author: Wang X
Year: 2006
Journal: Cell
Title: Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis.
Volume: 127
Issue: 4
Pages: 803-15
Strain
Attribute String: transgenic, mutant stock
Publication
First Author: Sharma S
Year: 2019
Journal: J Mol Histol
Title: Mapping the sites of localization of epithelial sodium channel (ENaC) and CFTR in segments of the mammalian epididymis.
Volume: 50
Issue: 2
Pages: 141-154
Ontology Term
Allele
Name: cystic fibrosis transmembrane conductance regulator; endonuclease-mediated mutation 3, Case Western Reserve University
Allele Type: Endonuclease-mediated
Attribute String: Hypomorph
Strain
Attribute String: coisogenic, endonuclease-mediated mutation, mutant strain
Genotype
Symbol: Cftr/Cftr
Background: C57BL/6J-Cftr/Cwr
Zygosity: hm
Has Mutant Allele: true
Publication
First Author: Rode B
Year: 2012
Journal: Hum Mol Genet
Title: The testis anion transporter TAT1 (SLC26A8) physically and functionally interacts with the cystic fibrosis transmembrane conductance regulator channel: a potential role during sperm capacitation.
Volume: 21
Issue: 6
Pages: 1287-98
Allele
Name: transgene insertion 6, University of North Carolina
Allele Type: Transgenic
Attribute String: Inserted expressed sequence
Publication
First Author: He J
Year: 2004
Journal: J Biol Chem
Title: Interaction with cystic fibrosis transmembrane conductance regulator-associated ligand (CAL) inhibits beta1-adrenergic receptor surface expression.
Volume: 279
Issue: 48
Pages: 50190-6
Protein
Organism: Mus musculus/domesticus
Length: 103  
Fragment?: true
Protein
Organism: Mus musculus/domesticus
Length: 166  
Fragment?: false
Publication
First Author: Soncini C
Year: 2001
Journal: Oncogene
Title: Ras-GAP SH3 domain binding protein (G3BP) is a modulator of USP10, a novel human ubiquitin specific protease.
Volume: 20
Issue: 29
Pages: 3869-79
Publication
First Author: Yuan J
Year: 2010
Journal: Cell
Title: USP10 regulates p53 localization and stability by deubiquitinating p53.
Volume: 140
Issue: 3
Pages: 384-96
Publication
First Author: Bomberger JM
Year: 2009
Journal: J Biol Chem
Title: The deubiquitinating enzyme USP10 regulates the post-endocytic sorting of cystic fibrosis transmembrane conductance regulator in airway epithelial cells.
Volume: 284
Issue: 28
Pages: 18778-89
Publication
First Author: Cheng J
Year: 2004
Journal: J Biol Chem
Title: Modulation of mature cystic fibrosis transmembrane regulator protein by the PDZ domain protein CAL.
Volume: 279
Issue: 3
Pages: 1892-8
Protein Domain
Type: Family
Description: Ubiquitin carboxyl-terminal hydrolase 10 (USP10) isa hydrolase that can remove conjugated ubiquitin from target proteins such as p53/TP53, BECN1, SNX3 and CFTR []. It regulates the localisation and stability of p53 by de-ubiquitinatiing p53 []. It is also implicated in vesicular transport and trafficking of membrane proteins [].
Protein Domain
Type: Family
Description: GOPC, also known as PIST or CAL, is primarily localized to the Golgi apparatus. It binds the G protein-coupled receptor beta1AR and modulates beta1AR intracellular trafficking []. GOPC also interacts with cystic fibrosis transmembrane regulator (CFTR), retains CFTR in the cell and targets it for degradation [].
Allele
Name: cystic fibrosis transmembrane conductance regulator; targeted mutation 1, University of Cambridge
Allele Type: Targeted
Attribute String: Null/knockout
Publication
First Author: Touré A
Year: 2007
Journal: Hum Mol Genet
Title: The testis anion transporter 1 (Slc26a8) is required for sperm terminal differentiation and male fertility in the mouse.
Volume: 16
Issue: 15
Pages: 1783-93
Publication
First Author: Toure A
Year: 2001
Journal: J Biol Chem
Title: Tat1, a novel sulfate transporter specifically expressed in human male germ cells and potentially linked to rhogtpase signaling.
Volume: 276
Issue: 23
Pages: 20309-15
Protein Domain
Type: Family
Description: The testis anion transporter TAT1 (solute carrier family 26 member 8; SLC26A8) mediates chloride, sulfate and oxalate transport and is specifically expressed in male germ cells and mature sperms [, ]. It is essential for correct sperm tail differentiation and motility, and hence male fertility []. TAT1 and CFTR (cystic fibrosis transmembrane conductance regulator) seem to cooperate in the regulation of the Cl-/HCO3- fluxes required for motility and capacitation of sperm [].
Publication
First Author: Pasyk S
Year: 2015
Journal: Proteomics
Title: The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation.
Volume: 15
Issue: 2-3
Pages: 447-61
Protein Domain
Type: Family
Description: The ABC transporter family is a group of membrane proteins that use the hydrolysis of ATP to power the translocation of a wide variety of substrates across cellular membranes. ABC transporters minimally consist of two conserved regions: a highly conserved nucleotide-binding domain (NBD) and a less conserved transmembrane domain (TMD). Eukaryotic ABC proteins are usually organised either as full transporters (containing two NBDs and two TMDs), or as half transporters (containing one NBD and one TMD), that have to form homo- or heterodimers in order to constitute a functional protein [].Cystic fibrosis transmembrane conductance regulator (CFTR, also known as ABCC7) is an eukaryotic protein belonging to the ABC-C subfamily of the ABC transporter family. CFTR protein is a chloride ion channel controlled by phosphorylation. It has a major role in electrolyte and fluid secretion. CFTR is important in the determination of fluid flow, ion concentration and transepithelial salttransport. Dysfunction of the CFTR channel causes the life-threateningdisease, cystic fibrosis, in which trans-epithelial ion transport is disrupted []. Defective phosphorylation has been seen to be a cause for this altered activity [].
Publication
First Author: Arora K
Year: 2015
Journal: Am J Pathol
Title: Altered cGMP dynamics at the plasma membrane contribute to diarrhea in ulcerative colitis.
Volume: 185
Issue: 10
Pages: 2790-804
Genotype
Symbol: Cftr/Cftr
Background: involves: 129S/SvEv
Zygosity: hm
Has Mutant Allele: true
HT Experiment  
Experiment Type: RNA-Seq
Study Type: Baseline
Source: GEO
Publication
First Author: Lohmann SM
Year: 1997
Journal: Trends Biochem Sci
Title: Distinct and specific functions of cGMP-dependent protein kinases.
Volume: 22
Issue: 8
Pages: 307-12
HT Experiment
Series Id: GSE33319
Experiment Type: transcription profiling by array
Study Type: WT vs. Mutant
Source: ArrayExpress
Publication
First Author: Groen A
Year: 2011
Journal: Gastroenterology
Title: Complementary functions of the flippase ATP8B1 and the floppase ABCB4 in maintaining canalicular membrane integrity.
Volume: 141
Issue: 5
Pages: 1927-37.e1-4
Protein
Organism: Mus musculus/domesticus
Length: 103  
Fragment?: true
Publication
First Author: Bull LN
Year: 1998
Journal: Nat Genet
Title: A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis.
Volume: 18
Issue: 3
Pages: 219-24
Publication
First Author: Verhulst PM
Year: 2010
Journal: Hepatology
Title: A flippase-independent function of ATP8B1, the protein affected in familial intrahepatic cholestasis type 1, is required for apical protein expression and microvillus formation in polarized epithelial cells.
Volume: 51
Issue: 6
Pages: 2049-60
Publication
First Author: Paulusma CC
Year: 2008
Journal: Hepatology
Title: ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity.
Volume: 47
Issue: 1
Pages: 268-78
Protein Domain
Type: Family
Description: Phospholipid-transporting ATPase IC (also known as ATP8B1 or ATPIC) belongs to subfamily IV of the P-type ATPases family, whose members transport phospholipids across the membrane. ATP8B1 can bind either CDC50A or CDC50B as its accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity [, , ].In hepatocytes, ATP8B1 localises in the canalicular membrane, where it may maintain the membrane integrity and to the function of ABCB4, an ABC floppase that play a role in bile export []. In the epithelial Caco-2 cells, ATP8B1 is required for apical protein expression and microvillus formation in polarised epithelial cells []. Mutations in ATP8B1 cause two forms of intrahepatic cholestasis, progressive familial intrahepatic cholestasis (PFIC) and benign recurrent intrahepatic cholestasis (BFIC) []. It is involved in the correct apical membrane localization of CDC42, CFTR and SLC10A2 [].
Publication
First Author: Schreiber R
Year: 2000
Journal: FEBS Lett
Title: Aquaporin 3 cloned from Xenopus laevis is regulated by the cystic fibrosis transmembrane conductance regulator.
Volume: 475
Issue: 3
Pages: 291-5
Publication
First Author: Wallis D
Year: 1994
Journal: J Hered
Title: Mapping the bovine homolog of the human cystic fibrosis gene.
Volume: 85
Issue: 6
Pages: 490-2
Publication
First Author: Gotoh T
Year: 2004
Journal: Cell Death Differ
Title: hsp70-DnaJ chaperone pair prevents nitric oxide- and CHOP-induced apoptosis by inhibiting translocation of Bax to mitochondria.
Volume: 11
Issue: 4
Pages: 390-402
Publication
First Author: Terada K
Year: 2000
Journal: J Biol Chem
Title: Human DnaJ homologs dj2 and dj3, and bag-1 are positive cochaperones of hsc70.
Volume: 275
Issue: 32
Pages: 24728-34
Publication
First Author: Lohi H
Year: 2002
Journal: J Biol Chem
Title: Functional characterization of three novel tissue-specific anion exchangers SLC26A7, -A8, and -A9.
Volume: 277
Issue: 16
Pages: 14246-54
Publication
First Author: Bryde S
Year: 2010
Journal: J Biol Chem
Title: CDC50 proteins are critical components of the human class-1 P4-ATPase transport machinery.
Volume: 285
Issue: 52
Pages: 40562-72
Publication
First Author: Stark JL
Year: 2014
Journal: Biochemistry
Title: Structure and function of human DnaJ homologue subfamily a member 1 (DNAJA1) and its relationship to pancreatic cancer.
Volume: 53
Issue: 8
Pages: 1360-72
Publication
First Author: Baaklini I
Year: 2020
Journal: Sci Rep
Title: Selective Binding of HSC70 and its Co-Chaperones to Structural Hotspots on CFTR.
Volume: 10
Issue: 1
Pages: 4176
Protein Domain
Type: Family
Description: The DnaJ proteins, also known as heat shock protein 40 (Hsp40 or Hsc40), are proteins originally identified in Escherichia coli that act as cochaperones to the molecular chaperone DnaK (Hsp70), which is responsible for several cellular processes such as rescuing misfolded proteins, folding polypeptide chains, transport of polypeptides through membranes, assembly and disassembly of protein complexes, and control of regulatory proteins [].Structurally, the DnaJ protein consists of an N-terminal conserved domain (called 'J' domain) of about 70 amino acids, a glycine-rich region ('G' domain') of about 30 residues, a central domain containing four repeats of a CXXCXGXG motif ('CRR' domain) and a C-terminal region of 120 to 170 residues.This entry represents a group of DnaJ domain containing proteins, including DNJA1/2/4 from humans, Scj1/Mas5/Xdj1/Apj1 from budding yeasts, and DNAJ2/3/19 from Arabidopsis. [].In humans, DNAJA1, DNAJA2 and HSC70 have been shown to play key roles in both the folding and degradation of wild-type and mutant CFTR (cystic fibrosis transmembrane conductance regulator) [, ]. They also function as co-chaperone for HSPA1B and negatively regulates the translocation of BAX from the cytosol to mitochondria in response to cellular stress, thereby protecting cells against apoptosis [].
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus caroli
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory