Research Topics
Genomes and Genes | RAYMOND FRIZZELLSummaryAffiliation: University of Pittsburgh Country: USA Publications
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Publications
Regulation of the epithelial sodium channel by membrane traffickingMichael B Butterworth
Dept of Cell Biology and Physiology, Univ of Pittsburgh, S375 BST, 3500 Terrace St, Pittsburgh, PA 15261, USA
Am J Physiol Renal Physiol 296:F10-24. 2009..While many details of the regulation of ENaC trafficking remain to be elucidated, knowledge of these mechanisms may provide further insights into ENaC activity in normal and disease states...
Physiology of epithelial chloride and fluid secretionRaymond A Frizzell
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
Cold Spring Harb Perspect Med 2:a009563. 2012....
The role of regulated CFTR trafficking in epithelial secretionCarol A Bertrand
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, S362 BST, 3500 Terrace St, Pittsburgh, PA 15261, USA
Am J Physiol Cell Physiol 285:C1-18. 2003..These models provide testable hypotheses that may lead to elucidation of CFTR trafficking mechanisms and permit their experimental manipulation in polarized epithelial cells...
Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulatorHui Zhang
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
J Biol Chem 277:28948-58. 2002....
Clathrin-mediated endocytosis of the epithelial sodium channel. Role of epsinHuamin Wang
Renal Electrolyte Division, Department of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania 15261, USA
J Biol Chem 281:14129-35. 2006..These results identify epsin as an accessory protein linking ENaC to the clathrin-based endocytic machinery thereby regulating the activity of this ion channel at the cell surface...
The deubiquitinating enzyme UCH-L3 regulates the apical membrane recycling of the epithelial sodium channelMichael B Butterworth
Department of Cell Biology and Physiology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15261, USA
J Biol Chem 282:37885-93. 2007..This constitutes a novel function for UCH in epithelia and in the regulation of ion channels and demonstrates the dynamic regulation of apically located ENaC by recycling, which is facilitated by this DUB...
Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epitheliaFei Sun
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA
FASEB J 22:3255-63. 2008..Thus, PTD-mediated DeltaFRD fragment delivery may provide a therapy for CF...
Regulation of CFTR trafficking by its R domainChristopher M Lewarchik
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
J Biol Chem 283:28401-12. 2008..These observations suggest that the NEG2 region at the C terminus of the R domain allows stabilization of CFTR in a regulated intracellular compartment from which it traffics to the plasma membrane in response to cAMP/PKA stimulation...
Rab11b regulates the apical recycling of the cystic fibrosis transmembrane conductance regulator in polarized intestinal epithelial cellsMark R Silvis
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, PA 15261, USA
Mol Biol Cell 20:2337-50. 2009..This is the first report detailing apical CFTR recycling in a native expression system and to demonstrate that Rab11b regulates apical recycling in polarized epithelial cells...
Acute ENaC stimulation by cAMP in a kidney cell line is mediated by exocytic insertion from a recycling channel poolMichael B Butterworth
Department of Cell Biology and Physiology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15261, USA
J Gen Physiol 125:81-101. 2005..The model supports the concept of ENaC recycling in response to repetitive cAMP stimulation...
SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epitheliaCarol A Bertrand
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA
J Gen Physiol 133:421-38. 2009....
Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesisHui Zhang
Department of Cell Biology and Physiology, University of Pittsburgh, Pittsburgh, Pennsylvania 15261, USA
J Biol Chem 281:11312-21. 2006....
Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutantsFei Sun
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
J Biol Chem 281:36856-63. 2006....
Syntaxin 1A regulates ENaC channel activitySteven B Condliffe
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15217, USA
J Biol Chem 279:10085-92. 2004..This rapid effect of S1A may modulate Na(+) entry rate during rapid increases in ENaC N...
AS160 modulates aldosterone-stimulated epithelial sodium channel forward traffickingXiubin Liang
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA
Mol Biol Cell 21:2024-33. 2010....
An obligatory heterodimer of 14-3-3beta and 14-3-3epsilon is required for aldosterone regulation of the epithelial sodium channelXiubin Liang
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
J Biol Chem 283:27418-25. 2008....
14-3-3 isoforms are induced by aldosterone and participate in its regulation of epithelial sodium channelsXiubin Liang
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, 3500 Terrace Street, Pittsburgh, PA 15261, USA
J Biol Chem 281:16323-32. 2006..Our studies show that aldosterone increases the expression of 14-3-3beta, which interacts with phospho-Nedd4-2 to block its interaction with ENaC, thus enhancing sodium absorption by increasing apical membrane ENaC density...
Cysteine string protein promotes proteasomal degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) by increasing its interaction with the C terminus of Hsp70-interacting protein and promoting CFTR ubiquitylationBela Z Schmidt
Department of Cell Biology and Physiology, University of Pittsburgh, Pittsburgh, Pennsylvania 15261
J Biol Chem 284:4168-78. 2009..These findings indicate that Csp not only regulates the exit of CFTR from the ER, but that this action is accompanied by Hsc70/Hsp70 and CHIP-mediated CFTR degradation...
Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradationAnnette Ahner
Department of Biological Sciences, University of Pittsburgh, Pittsburgh, PA 15260, USA
Mol Biol Cell 18:806-14. 2007....
Identification and membrane localization of electrogenic sodium bicarbonate cotransporters in Calu-3 cellsJames L Kreindler
Department of Pediatrics, Children s Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA
Biochim Biophys Acta 1762:704-10. 2006..These data suggest that these transporters contribute to regulated bicarbonate secretion across Calu-3 cells and perhaps human airway submucosal glands...
Syntaxin 1A regulates ENaC via domain-specific interactionsSteven B Condliffe
Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
J Biol Chem 278:12796-804. 2003..This study provides support for the concept that S1A inhibits ENaC-mediated Na(+) transport by decreasing cell surface channel number via direct protein-protein interactions at the ENaC C termini...
Transepithelial impedance analysis of chloride secretionAshvani K Singh
Department of Cell Biology and Physiology, University of Pittsburgh, Pittsburgh, PA, USA
Methods Mol Med 70:129-42. 2002
Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosisMike M Myerburg
Division of Pulmonary, Allergy, and Critical Care Medicine, Univ of Pittsburgh Medical Center, 628 NW Montefiore Univ Hospital, 3459 Fifth Ave, Pittsburgh, PA 15213, USA
Am J Physiol Lung Cell Mol Physiol 294:L932-41. 2008....
The epithelial sodium channel (ENaC) traffics to apical membrane in lipid rafts in mouse cortical collecting duct cellsWarren G Hill
Division of Matrix Biology, Department of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA
J Biol Chem 282:37402-11. 2007..We conclude that ENaC is present in and regulated by lipid rafts. The data are consistent with a model in which rafts mediate the constitutive apical delivery of ENaC...
Regulation of human cystic fibrosis transmembrane conductance regulator (CFTR) by serum- and glucocorticoid-inducible kinase (SGK1)J Denry Sato
Mount Desert Island Biological Laboratory, Salisbury Cove, USA
Cell Physiol Biochem 20:91-8. 2007..Because SGK increases the plasma membrane expression of other ion channels, the goal of this paper was to test the hypothesis that SGK1 stimulates CFTR Cl currents by increasing the number of CFTR Cl channels in the plasma membrane...
Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: a mechanism for sodium hyperabsorption in cystic fibrosisMike M Myerburg
Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, Pennsylvania 15213, USA
J Biol Chem 281:27942-9. 2006....
Finally, mice with CF lung diseaseRaymond A Frizzell
Nat Med 10:452-4. 2004
Research Grants
- Chaperone Actions in CFTR BiogenesisRaymond A Frizzell; Fiscal Year: 2010..This novel concept, that sHsps catalyze the transfer of SUMO to their substrates, will be evaluated as a general mechanism of sHsp chaperone function. ..
- TRAFFIC REGULATORY PROTEINS AND ENACRaymond A Frizzell; Fiscal Year: 2010..This work is expected to reveal new regulators and therapeutic targets for the control of sodium and fluid transport in the kidney. ..
- TRAFFIC REGULATORY PROTEINS AND ENACRAYMOND FRIZZELL; Fiscal Year: 2009..These studies will determine how ENaC's interactions with traffic regulatory proteins controls apical membrane channel density under normal conditions and in response to physiological modulators of Na transport. ..
- Co-chaperone Actions in CFTR BiogenesisRAYMOND FRIZZELL; Fiscal Year: 2007..Understanding the role of direct, high affinity interactions with Csp may identify targets for favorable and selective modulation of CFTR biogenesis. ..
- TRAFFIC REGULATORY PROTEINS AND ENACRAYMOND FRIZZELL; Fiscal Year: 2007..These studies will determine how ENaC's interactions with traffic regulatory proteins controls apical membrane channel density under normal conditions and in response to physiological modulators of Na transport. ..
- TRAFFIC REGULATORY PROTEINS AND ENACRAYMOND FRIZZELL; Fiscal Year: 2002..This process has important implications for the regulation of body salt and water homeostasis, the control of blood pressure and cardiovascular diseases. ..
- Chaperone Actions in CFTR BiogenesisRaymond A Frizzell; Fiscal Year: 2010..This novel concept, that sHsps catalyze the transfer of SUMO to their substrates, will be evaluated as a general mechanism of sHsp chaperone function. ..
