A Genomic Signature Approach to Rescue ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator Biosynthesis and Function

Por Ramachandran, Shyam, Osterhaus, Samantha R., Karp, Philip H., Welsh, Michael J., McCray, Paul B.
Publicado em 2014

Relationships among CFTR expression, HCO(3)(−) secretion, and host defense may inform gene- and cell-based cystic fibrosis therapies

Por Shah, Viral S., Ernst, Sarah, Tang, Xiao Xiao, Karp, Philip H., Parker, Connor P., Ostedgaard, Lynda S., Welsh, Michael J.
Publicado no Proc Natl Acad Sci U S A (2016)

Isoform-Specific Regulation and Localization of the Coxsackie and Adenovirus Receptor in Human Airway Epithelia

Por Excoffon, Katherine J. D. A., Gansemer, Nicholas D., Mobily, Matthew E., Karp, Philip H., Parekh, Kalpaj R., Zabner, Joseph
Publicado em 2010

Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR(−/−) mice

Por Ostedgaard, Lynda S., Meyerholz, David K., Vermeer, Daniel W., Karp, Philip H., Schneider, Lindsey, Sigmund, Curt D., Welsh, Michael J.
Publicado em 2011

CFTR with a partially deleted R domain corrects the cystic fibrosis chloride transport defect in human airway epithelia in vitro and in mouse nasal mucosa in vivo

Por Ostedgaard, Lynda S., Zabner, Joseph, Vermeer, Daniel W., Rokhlina, Tatiana, Karp, Philip H., Stecenko, Arlene A., Randak, Christoph, Welsh, Michael J.
Publicado em 2002

Processing and function of CFTR-ΔF508 are species-dependent

Por Ostedgaard, Lynda S., Rogers, Christopher S., Dong, Qian, Randak, Christoph O., Vermeer, Daniel W., Rokhlina, Tatiana, Karp, Philip H., Welsh, Michael J.
Publicado em 2007

Chemosensory Functions for Pulmonary Neuroendocrine Cells

Por Gu, Xiaoling, Karp, Philip H., Brody, Steven L., Pierce, Richard A., Welsh, Michael J., Holtzman, Michael J., Ben-Shahar, Yehuda
Publicado em 2014

The osmolyte xylitol reduces the salt concentration of airway surface liquid and may enhance bacterial killing

Por Zabner, Joseph, Seiler, Michael P., Launspach, Janice L., Karp, Philip H., Kearney, William R., Look, Dwight C., Smith, Jeffrey J., Welsh, Michael J.
Publicado em 2000

Human cystic fibrosis airway epithelia have reduced Cl(−) conductance but not increased Na(+) conductance

Por Itani, Omar A., Chen, Jeng-Haur, Karp, Philip H., Ernst, Sarah, Keshavjee, Shaf, Parekh, Kalpaj, Klesney-Tait, Julia, Zabner, Joseph, Welsh, Michael J.
Publicado em 2011

A shortened adeno-associated virus expression cassette for CFTR gene transfer to cystic fibrosis airway epithelia

Por Ostedgaard, Lynda S., Rokhlina, Tatiana, Karp, Philip H., Lashmit, Philip, Afione, Sandra, Schmidt, Michael, Zabner, Joseph, Stinski, Mark F., Chiorini, Jay A., Welsh, Michael J.
Publicado em 2005

Adenoviral Gene Transfer Corrects the Ion Transport Defect in the Sinus Epithelia of a Porcine CF Model

Por Potash, Andrea E, Wallen, Tanner J, Karp, Philip H, Ernst, Sarah, Moninger, Thomas O, Gansemer, Nicholas D, Stoltz, David A, Zabner, Joseph, Chang, Eugene H
Publicado em 2013

Acidic pH increases airway surface liquid viscosity in cystic fibrosis

Por Tang, Xiao Xiao, Ostedgaard, Lynda S., Hoegger, Mark J., Moninger, Thomas O., Karp, Philip H., McMenimen, James D., Choudhury, Biswa, Varki, Ajit, Stoltz, David A., Welsh, Michael J.
Publicado no J Clin Invest (2016)

Integrin α6β4 Identifies Human Distal Lung Epithelial Progenitor Cells with Potential as a Cell-Based Therapy for Cystic Fibrosis Lung Disease

Por Li, Xiaopeng, Rossen, Nathan, Sinn, Patrick L., Hornick, Andrew L., Steines, Benjamin R., Karp, Philip H., Ernst, Sarah E., Adam, Ryan J., Moninger, Thomas O., Levasseur, Dana N., Zabner, Joseph
Publicado em 2013

Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia

Por Chen, Jeng-Haur, Stoltz, David A., Karp, Philip H., Ernst, Sarah E., Pezzulo, Alejandro A., Moninger, Thomas O., Rector, Michael V., Reznikov, Leah R., Launspach, Janice L., Chaloner, Kathryn, Zabner, Joseph, Welsh, Michael J.
Publicado em 2010

Post-Transcriptional Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Expression and Function by MicroRNAs

Por Ramachandran, Shyam, Karp, Philip H., Osterhaus, Samantha R., Jiang, Peng, Wohlford-Lenane, Christine, Lennox, Kim A., Jacobi, Ashley M., Praekh, Kal, Rose, Scott D., Behlke, Mark A., Xing, Yi, Welsh, Michael J., McCray, Paul B.
Publicado em 2013

A microRNA network regulates expression and biosynthesis of wild-type and ΔF508 mutant cystic fibrosis transmembrane conductance regulator

Por Ramachandran, Shyam, Karp, Philip H., Jiang, Peng, Ostedgaard, Lynda S., Walz, Amy E., Fisher, John T., Keshavjee, Shaf, Lennox, Kim A., Jacobi, Ashley M., Rose, Scott D., Behlke, Mark A., Welsh, Michael J., Xing, Yi, McCray, Paul B.
Publicado em 2012

Medical reversal of chronic rhinosinusitis in a cystic fibrosis patient with ivacaftor

Por Chang, Eugene H., Tang, Xiao Xiao, Shah, Viral S., Launspach, Janice L., Ernst, Sarah E., Hilkin, Brieanna, Karp, Philip H., Abou Alaiwa, Mahmoud H., Graham, Scott M., Hornick, Douglas B., Welsh, Michael J., Stoltz, David A., Zabner, Joseph
Publicado no Int Forum Allergy Rhinol (2014)

CFTR is required for maximal transepithelial liquid transport in pig alveolar epithelia

Por Li, Xiaopeng, Comellas, Alejandro P., Karp, Philip H., Ernst, Sarah E., Moninger, Thomas O., Gansemer, Nicholas D., Taft, Peter J., Pezzulo, Alejandro A., Rector, Michael V., Rossen, Nathan, Stoltz, David A., McCray, Paul B., Welsh, Michael J., Zabner, Joseph
Publicado em 2012

Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense

Por Li, Xiaopeng, Tang, Xiao Xiao, Vargas Buonfiglio, Luis G., Comellas, Alejandro P., Thornell, Ian M., Ramachandran, Shyam, Karp, Philip H., Taft, Peter J., Sheets, Kelsey, Abou Alaiwa, Mahmoud H., Welsh, Michael J., Meyerholz, David K., Stoltz, David A., Zabner, Joseph
Publicado no Am J Physiol Lung Cell Mol Physiol (2016)

Sinus Hypoplasia Precedes Sinus Infection in a Porcine Model of Cystic Fibrosis

Por Chang, Eugene H, Pezzulo, Alejandro A, Meyerholz, David K, Potash, Andrea E, Wallen, Tanner J, Reznikov, Leah R, Sieren, Jessica C, Karp, Philip H, Ernst, Sarah, Moninger, Thomas O, Gansemer, Nicholas D, McCray, Paul B, Stoltz, David A, Welsh, Michael J, Zabner, Joseph
Publicado em 2012