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List of works by William B Guggino

(Na+ + K+)-ATPase is a target for phosphoinositide 3-kinase/protein kinase B and protein kinase C pathways triggered by albumin.

scientific article

49 Assays of dynamics, mechanisms, and regulation of ATP transport and release: Implications for study of ABC transporter function

scientific article published on January 1, 1998

A 59 amino acid insertion increases Ca(2+) sensitivity of rbslo1, a Ca2+ -activated K(+) channel in renal epithelia.

scientific article

A Golgi-associated PDZ Domain Protein Modulates Cystic Fibrosis Transmembrane Regulator Plasma Membrane Expression ⬇️

scientific article published on November 13, 2001

A Novel Role of Protein Tyrosine Kinase2 in Mediating Chloride Secretion in Human Airway Epithelial Cells ⬇️

scientific article published on July 13, 2011

A PDZ-interacting domain in CFTR is an apical membrane polarization signal

scientific article

A Preclinical Study in Rhesus Macaques for Cystic Fibrosis to Assess Gene Transfer and Transduction by AAV1 and AAV5 with a Dual-Luciferase Reporter System.

scientific article published on 20 July 2017

A delayed rectifier potassium current in Xenopus oocytes

scientific article

A functional CFTR-NBF1 is required for ROMK2-CFTR interaction ⬇️

scientific article published on November 1, 1997

A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis ⬇️

scientific article published on 01 April 1998

A new role for heat shock factor 27 in the pathophysiology of Clostridium difficile toxin B

scientific article published on 11 November 2019

A novel gamma-aminobutyric acid receptor subunit (rho 2) cloned from human retina forms bicuculline-insensitive homooligomeric receptors in Xenopus oocytes

scientific article published on November 1994

A phase I study of an adeno-associated virus-CFTR gene vector in adult CF patients with mild lung disease

scientific article published on June 1996

A phase I trial of intranasal Moli1901 for cystic fibrosis

scientific article

A phase I/II study of tgAAV-CF for the treatment of chronic sinusitis in patients with cystic fibrosis

scientific article published on 01 April 1998

A phase II, double-blind, randomized, placebo-controlled clinical trial of tgAAVCF using maxillary sinus delivery in patients with cystic fibrosis with antrostomies

scientific article published in July 2002

A regulatory role of polycystin-1 on cystic fibrosis transmembrane conductance regulator plasma membrane expression

scientific article published on 14 August 2006

A role for CFTR in human autosomal dominant polycystic kidney disease

scientific article published on 01 January 1996

A single amino acid in gamma-aminobutyric acid rho 1 receptors affects competitive and noncompetitive components of picrotoxin inhibition

scientific article

A single histidine residue is essential for zinc inhibition of GABA rho 1 receptors

scientific article

Absence of ClC5 in knockout mice leads to glycosuria, impaired renal glucose handling and low proximal tubule GLUT2 protein expression

scientific article published in January 2007

Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity

scientific article (publication date: 29 September 2000)

Adeno-Associated Virus (AAV) gene therapy for cystic fibrosis: current barriers and recent developments

scientific article published on 28 June 2017

Adenylyl cyclase is involved in desensitization and recovery of ATP-stimulated Cl−secretion in MDCK cells ⬇️

scientific article published on February 1, 1998

Albumin endocytosis in proximal tubule cells is modulated by angiotensin II through an AT2 receptor-mediated protein kinase B activation

scientific article

Alterations in a voltage-gated K+ current during the differentiation of ML-1 human myeloblastic leukemia cells.

scientific article published in March 1993

Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator

scientific article published on May 1995

Amiloride-sensitive sodium channels contribute to the woes of the flu.

scientific article

Analysis of ClC-2 channels as an alternative pathway for chloride conduction in cystic fibrosis airway cells.

scientific article

Anthracene-9-carboxylic acid inhibits renal chloride reabsorption

scientific article published on 01 July 1983

Antibody and Reverse Capture Protein Microarrays for Clinical Proteomics

Apical and basolateral ATP stimulates tracheal epithelial chloride secretion via multiple purinergic receptors

scientific article published on 01 June 1996

Aquaporin CHIP: the archetypal molecular water channel ⬇️

scientific article published on October 1, 1993

Atrial natriuretic peptide modulates cystic fibrosis transmembrane conductance regulator chloride channel expression in rat proximal colon and human intestinal epithelial cells

scientific journal article

Basolateral chloride transporters in autosomal dominant polycystic kidney disease

scientific article published on 01 August 2002

Biological Differences in rAAV Transduction of Airway Epithelia in Humans and in Old World Non-human Primates.

scientific article

Bioluminescence detection of ATP release mechanisms in epithelia

scientific article published on November 1998

Blocking agents of Ca2+-activated K+ channels in cultured medullary thick ascending limb cells

scientific article published on 01 February 1987

Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents

scientific article published on May 1994

Both the wild type and a functional isoform of CFTR are expressed in kidney

scientific article published in June 1996

CFTR Controls the Activity of NF-κB by Enhancing the Degradation of TRADD.

scientific article published on 14 December 2016

CFTR Folding Consortium: methods available for studies of CFTR folding and correction

scientific article published on January 2011

CFTR gene transduction in neonatal rabbits using anadeno-associated virus (AAV) vector ⬇️

scientific article published on May 1, 1997

CFTR is a conductance regulator as well as a chloride channel

scientific article

CFTR mediates electrogenic chloride secretion in mouse inner medullary collecting duct (mIMCD-K2) cells

scientific article

CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP

scientific article

CIC-2: a developmentally dependent chloride channel expressed in the fetal lung and downregulated after birth.

scientific article

CORRESPONDENCE

Ca2+-activated K+ channels from cultured renal medullary thick ascending limb cells: effects of pH

scientific article published on 01 August 1989

Ca2+-activated K+ channels in cultured medullary thick ascending limb cells

scientific article published on 01 February 1987

Cadmium impairs albumin reabsorption by down-regulating megalin and ClC5 channels in renal proximal tubule cells.

scientific article

Cellular and paracellular resistances of the Necturus proximal tubule

scientific article published on 01 January 1982

Characterization of aquaporin-6 as a nitrate channel in mammalian cells. Requirement of pore-lining residue threonine 63

scientific journal article

Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator ⬇️

scientific article published on March 3, 1998

Chloride channels in the kidney: lessons learned from knockout animals

scientific article

Chloride transport across the basolateral cell membrane of theNecturus proximal tubule: Dependence on bicarbonate and sodium ⬇️

scientific article published on 01 January 1983

Cl- channels in CF: lack of activation by protein kinase C and cAMP-dependent protein kinase

scientific article published on June 1989

ClC-5: ontogeny of an alternative chloride channel in respiratory epithelia

scientific article published on 01 March 2002

ClC-5: role in endocytosis in the proximal tubule.

scientific article published on 7 June 2005

Cloning and characterization of maxi K+channel α-subunit in rabbit kidney ⬇️

scientific article published on October 1, 1997

Cloning and expression of two brain-specific inwardly rectifying potassium channels

scientific article

Cloning of a putative human voltage-gated chloride channel (CIC-2) cDNA widely expressed in human tissues

scientific article

Cloning of the gamma-aminobutyric acid (GABA) rho 1 cDNA: a GABA receptor subunit highly expressed in the retina ⬇️

scientific article published on April 1, 1991

Cofilin interacts with ClC-5 and regulates albumin uptake in proximal tubule cell lines.

scientific article

Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27.

scientific article

Complement yourself: Transcomplementation rescues partially folded mutant proteins

scientific article

Continuous detection of extracellular ATP on living cells by using atomic force microscopy

scientific article published on October 1999

Correcting the cystic fibrosis disease mutant, A455E CFTR

scientific article published in 2014

Correctors Rescue CFTR Mutations in Nucleotide-Binding Domain 1 (NBD1) by Modulating Proteostasis.

scientific article published on 11 February 2016

Correlation between DNA transfer and cystic fibrosis airway epithelial cell correction after recombinant adeno-associated virus serotype 2 gene therapy

scientific article

Cystic fibrosis transmembrane conductance regulator-associated ATP release is controlled by a chloride sensor

scientific article

Cystic fibrosis transmembrane regulator missing the first four transmembrane segments increases wild type and DeltaF508 processing

scientific article published on 28 May 2008

De novo biosynthetic profiling of high abundance proteins in cystic fibrosis lung epithelial cells

scientific article

Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway

scientific article published on 21 March 2004

Deposition and expression of aerosolized rAAV vectors in the lungs of Rhesus macaques

scientific article published on 01 October 2002

Determinants of AQP6 trafficking to intracellular sites versus the plasma membrane in transfected mammalian cells

scientific article

Developmental regulation of CFTR expression during human nephrogenesis

scientific article published in September 1996

Dexamethasone Regulates CFTR Expression in Calu-3 Cells with the Involvement of Chaperones HSP70 and HSP90 ⬇️

scientific article published on December 13, 2012

Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells

scientific article published on January 1995

Direct modulation of secretory chloride channels by arachidonic and other cis unsaturated fatty acids ⬇️

scientific article published on August 1, 1990

Does the use of recombinant AAV2 in pulmonary gene therapy damage lung function?

scientific article published on 14 September 2007

Drosophila TRPA1 channel is required to avoid the naturally occurring insect repellent citronellal

scientific article

Drosophila TRPA1 channel mediates chemical avoidance in gustatory receptor neurons.

scientific article

Dual reporter comparative indexing of rAAV pseudotyped vectors in chimpanzee airway.

scientific article published on 13 October 2009

Effect of adeno-associated virus-specific immunoglobulin G in human amniotic fluid on gene transfer.

scientific article published on March 2003

Electrical effects of potassium and bicarbonate on proximal tubule cells of Necturus

scientific article published on 01 January 1984

Epac1 mediates protein kinase A-independent mechanism of forskolin-activated intestinal chloride secretion

scientific article

Estrogen modulates ClC-2 chloride channel gene expression in rat kidney.

scientific article

Expression of CFTR in human and bovine thyroid epithelium

scientific article published on 01 April 1997

Expression of a truncated cystic fibrosis transmembrane conductance regulator with an AAV5-pseudotyped vector in primates

scientific article published on 13 February 2007

Forskolin and antidiuretic hormone stimulate a Ca2+-activated K+ channel in cultured kidney cells

scientific article published in September 1985

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

scientific article published on February 2016

Functional characterization of a recombinant adeno-associated virus 5-pseudotyped cystic fibrosis transmembrane conductance regulator vector

scientific article published on September 2004

Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report

scientific article

G protein G alpha i-2 inhibits outwardly rectifying chloride channels in human airway epithelial cells

scientific article published on August 1995

GABA rho 2 receptor pharmacological profile: GABA recognition site similarities to rho 1

scientific article

Gender dependence for a subset of the low-abundance signaling proteome in human platelets ⬇️

scientific article published on April 13, 2010

Gene expression profile analysis of 4-phenylbutyrate treatment of IB3-1 bronchial epithelial cell line demonstrates a major influence on heat-shock proteins ⬇️

scientific article published on January 15, 2004

Gene therapy for cystic fibrosis paved the way for the use of AAV in gene therapy

scientific article published on 13 April 2020

Gene transfer by lipofection in rabbit and human secretory epithelial cells.

scientific article

Generation and Genetic Engineering of Human Induced Pluripotent Stem Cells Using Designed Zinc Finger Nucleases ⬇️

scientific article published on October 19, 2012

Gout-causing Q141K mutation in ABCG2 leads to instability of the nucleotide-binding domain and can be corrected with small molecules ⬇️

scientific article published on March 14, 2013

High citrate diet delays progression of renal insufficiency in the ClC-5 knockout mouse model of Dent's disease

scientific article

Histone deacetylase 6 inhibition reduces cysts by decreasing cAMP and Ca2+ in knock-out mouse models of polycystic kidney disease.

scientific article published on 8 September 2017

Hypoxia-induced mitogenic factor/FIZZ1 induces intracellular calcium release through the PLC-IP(3) pathway

scientific article published on 08 May 2009

Identification of a polycystin-1 cleavage product, P100, that regulates store operated Ca entry through interactions with STIM1.

scientific article

Identification of a urate transporter, ABCG2, with a common functional polymorphism causing gout

scholarly article

Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure

scientific article published on 3 November 2004

Impaired acidification in early endosomes of ClC-5 deficient proximal tubule

scientific article

Inhibition of histone deacetylase 6 activity reduces cyst growth in polycystic kidney disease

scientific article published on 25 March 2016

Insulin-Like Growth Factor 1 (IGF-1) Enhances the Protein Expression of CFTR ⬇️

scientific article published on March 28, 2013

Intracellular cysteines of the cystic fibrosis transmembrane conductance regulator (CFTR) modulate channel gating

scientific article published on January 2002

Ion permeation of AQP6 water channel protein. Single channel recordings after Hg2+ activation

scientific article published on 28 May 2002

Isomeric yohimbine alkaloids block calcium-activated K+ channels in medullary thick ascending limb cells of rabbit kidney

scientific article published on 01 January 1989

Localization studies of rare missense mutations in cystic fibrosis transmembrane conductance regulator (CFTR) facilitate interpretation of genotype-phenotype relationships.

scientific article

Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules

scientific journal article

Lung gene therapy with highly compacted DNA nanoparticles that overcome the mucus barrier

scientific article published on 14 January 2014

Mechanism of distal tubular chloride transport in Amphiuma kidney

scientific article published on 01 April 1982

Membrane Trafficking of the Cystic Fibrosis Gene Product, Cystic Fibrosis Transmembrane Conductance Regulator, Tagged with Green Fluorescent Protein in Madin-Darby Canine Kidney Cells ⬇️

scientific article published on August 21, 1998

Membrane topology of aquaporin CHIP. Analysis of functional epitope-scanning mutants by vectorial proteolysis

scientific article

Membrane-associated heparan sulfate is not required for rAAV-2 infection of human respiratory epithelia

scientific article published on 22 April 2006

Methods to Monitor Cell Surface Expression and Endocytic Trafficking of CFTR in Polarized Epithelial Cells ⬇️

scientific article published on January 1, 2011

Mis-regulation of mammalian target of rapamycin (mTOR) complexes induced by albuminuria in proximal tubules

scientific article

Modification of Ca2+-activated K+ channels in cultured medullary thick ascending limb cells by N-bromoacetamide

scientific article published in January 1987

Modulation of ion transport in cultured rabbit tracheal epithelium by lipoxygenase metabolites

scientific article published on November 1, 1992

Modulation of mature cystic fibrosis transmembrane regulator protein by the PDZ domain protein CAL

scientific article (publication date: 16 January 2004)

Molecular characterization of an aquaporin cDNA from brain: candidate osmoreceptor and regulator of water balance

scientific article

Molecular cloning and characterization of AqpZ, a water channel from Escherichia coli

scientific article published on December 1995

Molecular structure of the water channel through aquaporin CHIP. The hourglass model

scientific article

Multiple sequences in the C terminus of MaxiK channels are involved in expression, movement to the cell surface, and apical localization

scholarly article

Nedd4-2 functionally interacts with ClC-5: involvement in constitutive albumin endocytosis in proximal tubule cells

scientific article

Overcoming the cystic fibrosis sputum barrier to leading adeno-associated virus gene therapy vectors

scientific article

PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator

scientific article published on 7 August 2002

PKB and megalin determine the survival or death of renal proximal tubule cells

scholarly article

PTH regulates expression of ClC-5 chloride channel in the kidney

scientific article published on 01 February 2000

Peptide binding consensus of the NHE‐RF‐PDZ1 domain matches the C‐terminal sequence of cystic fibrosis transmembrane conductance regulator (CFTR) ⬇️

scientific article published on May 1, 1998

Phase I trial of intranasal and endobronchial administration of a recombinant adeno-associated virus serotype 2 (rAAV2)-CFTR vector in adult cystic fibrosis patients: a two-part clinical study

scientific article published in July 2003

Polycystin 2 interacts with type I inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling

scientific article

Polycystin-1 interacts with inositol 1,4,5-trisphosphate receptor to modulate intracellular Ca2+ signaling with implications for polycystic kidney disease

scientific article published on 23 October 2009

Polycystin-1 negatively regulates Polycystin-2 expression via the aggresome/autophagosome pathway.

scientific article published on 23 January 2014

Polycystin-1, 2, and STIM1 interact with IP(3)R to modulate ER Ca release through the PI3K/Akt pathway

scientific article published on 17 June 2011

Potassium transport in the early distal tubule of Amphiuma kidney. Effects of potassium adaptation

scientific article published on 01 March 1983

Protein microarray platforms for clinical proteomics.

scientific article published on 10 August 2007

Rapid gating and anion permeability of an intracellular aquaporin

scientific article published on November 1999

Regulation of CFTR Expression and Arginine Vasopressin Activity Are Dependent on Polycystin-1 in Kidney-Derived Cells.

scientific article published on 8 January 2016

Regulation of cystic fibrosis transmembrane conductance regulator (CFTR) gene transcription and alternative RNA splicing in a model of developing intestinal epithelium ⬇️

scientific article published on September 25, 1992

Regulation of cystic fibrosis transmembrane regulator trafficking and protein expression by a Rho family small GTPase TC10

scientific article

Regulation of intracellular chloride activity during perfusion with hypertonic solutions in the Necturus proximal tubule

scientific article published on 01 January 1983

Regulation of the basolateral potassium conductance of theNecturus proximal tubule ⬇️

scientific article published on 01 January 1984

Renal anion transport

scientific article published on September 1989

Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome

scientific article published on 20 June 2018

Rescue of NBD2 mutants N1303K and S1235R of CFTR by small-molecule correctors and transcomplementation

scientific article

Rescuing Trafficking Mutants of the ATP-binding Cassette Protein, ABCA4, with Small Molecule Correctors as a Treatment for Stargardt Eye Disease.

scientific article published on 19 June 2015

Resistance properties of the diluting segment of Amphiuma kidney: influence of potassium adaptation.

scientific article

Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease.

scientific article published on 13 April 2006

Restoration of F508-del Function by Transcomplementation: The Partners Meet in the Endoplasmic Reticulum

scientific article published on 01 January 2019

Role of calcium in adult onset polycystic kidney disease

article

Role of intracellular calcium in volume regulation by rabbit medullary thick ascending limb cells

scientific article published on 01 March 1991

Role of purinergic receptors in chloride secretion in Caco-2 cells ⬇️

scientific article published on June 1, 1997

STIM1fl/fl Ksp-Cre Mouse has Impaired Renal Water Balance

scientific article published on 24 June 2016

Screening for CLCN5 mutation in renal calcium stone formers patients

scientific article published on 2 February 2005

Sensitivity of a renal K+ channel (ROMK2) to the inhibitory sulfonylurea compound glibenclamide is enhanced by coexpression with the ATP-binding cassette transporter cystic fibrosis transmembrane regulator

scientific article published on July 1996

Sequences in the Amino Termini of GABA ρ and GABAA Subunits Specify Their Selective Interaction In Vitro ⬇️

scientific article published on January 1, 1998

Small nuclear RNAs U11 and U12 modulate expression of TNR-CFTR mRNA in mammalian kidneys

scientific article

Spiperone, identified through compound screening, activates calcium-dependent chloride secretion in the airway

scientific article

Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator

scientific article

Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant

scientific article

Successful transgene expression with serial doses of aerosolized rAAV2 vectors in rhesus macaques

scientific article

Syntaxin 6 and CAL mediate the degradation of the cystic fibrosis transmembrane conductance regulator

scientific article

Syntaxin 8 and the Endoplasmic Reticulum Processing of ΔF508-CFTR

scientific article published on 28 November 2018

Targeting CAL as a negative regulator of DeltaF508-CFTR cell-surface expression: an RNA interference and structure-based mutagenetic approach

scientific article published on 11 December 2006

The CFTR-Associated Ligand Arrests the Trafficking of the Mutant ΔF508 CFTR Channel in the ER Contributing to Cystic Fibrosis

scientific article published on 29 January 2018

The ClC-5 knockout mouse model of Dent's disease has renal hypercalciuria and increased bone turnover

scientific article published in April 2003

The Hypertonic Environment Differentially Regulates Wild-type CFTR and TNR-CFTR Chloride Channels ⬇️

scientific article published on October 29, 2010

The N-terminal domain of human GABA receptor rho1 subunits contains signals for homooligomeric and heterooligomeric interaction

scientific article (publication date: 23 May 1997)

The PDZ-interacting domain of cystic fibrosis transmembrane conductance regulator is required for functional expression in the apical plasma membrane

scientific article (publication date: September 2000)

The cytoplasmic tail of large conductance, voltage- and Ca2+-activated K+ (MaxiK) channel is necessary for its cell surface expression

scientific article published on 15 November 2002

The effect of furosemide on luminal sodium, chloride and potassium transport in the early distal tubule of Amphiuma kidney. Effects of potassium adaptation

scientific article published on 01 January 1983

The loss of the chloride channel, ClC-5, delays apical iodide efflux and induces a euthyroid goiter in the mouse thyroid gland

scientific article

The mercury-sensitive residue at cysteine 189 in the CHIP28 water channel ⬇️

scientific article published on January 5, 1993

Transcomplementation by a Truncation Mutant of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Enhances ΔF508 Processing through a Biomolecular Interaction ⬇️

scientific article published on March 5, 2013

Transcriptional adaptation to Clcn5 knockout in proximal tubules of mouse kidney.

scientific article

Transduction of Surface and Basal Cells in Rhesus Macaque Lung Following Repeat Dosing with AAV1CFTR

scientific article published on 01 September 2020

Transmembrane Mutations Alter the Channel Characteristics of the Cystic Fibrosis Transmembrane Conductance Regulator Expressed in Xenopus Oocytes

article

Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents

scientific article

Ubiquitination and degradation of CFTR by the E3 ubiquitin ligase MARCH2 through its association with adaptor proteins CAL and STX6

scientific article

Volume regulation in the early proximal tubule of the Necturus kidney.

scientific article

WNK4 enhances the degradation of NCC through a sortilin-mediated lysosomal pathway

scientific article

WNK4 kinase regulates surface expression of the human sodium chloride cotransporter in mammalian cells

scientific article

Water channel properties of major intrinsic protein of lens

scientific article

Wireless control of cellular function by activation of a novel protein responsive to electromagnetic fields.

scientific article

cGMP stimulates sodium and chloride currents in rat tracheal airway epithelia.

scientific article published on March 1997

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