Search Results - "Lee, Po‐Shun"

Orkambi® and amplifier co‐therapy improves function from a rare CFTR mutation in gene‐edited cells and patient tissue by Molinski, Steven V, Ahmadi, Saumel, Ip, Wan, Ouyang, Hong, Villella, Adriana, Miller, John P, Lee, Po‐Shun, Kulleperuma, Kethika, Du, Kai, Di Paola, Michelle, Eckford, Paul DW, Laselva, Onofrio, Huan, Ling Jun, Wellhauser, Leigh, Li, Ellen, Ray, Peter N, Pomès, Régis, Moraes, Theo J, Gonska, Tanja, Ratjen, Felix, Bear, Christine E

“…The combination therapy of lumacaftor and ivacaftor (Orkambi ® ) is approved for patients bearing the major cystic fibrosis (CF) mutation: ΔF508 . It has been…”
Get full text

Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D- CFTR mutation and preserved spirometry: a randomised controlled trial by Davies, Jane, Prof, Sheridan, Helen, MB ChB, Bell, Nicholas, MB ChB, Cunningham, Steve, PhD, Davis, Stephanie D, Prof, Elborn, J Stuart, Prof, Milla, Carlos E, MD, Starner, Timothy D, MD, Weiner, Daniel J, MD, Lee, Po-Shun, MD, Ratjen, Felix, Prof

“…Summary Background Ivacaftor has shown a clinical benefit in patients with cystic fibrosis who have the G551D- CFTR mutation and reduced lung function. Lung…”
Get full text

Estradiol and mTORC2 cooperate to enhance prostaglandin biosynthesis and tumorigenesis in TSC2-deficient LAM cells by Li, Chenggang, Lee, Po-Shun, Sun, Yang, Gu, Xiaoxiao, Zhang, Erik, Guo, Yanan, Wu, Chin-Lee, Auricchio, Neil, Priolo, Carmen, Li, Jing, Csibi, Alfredo, Parkhitko, Andrey, Morrison, Tasha, Planaguma, Anna, Kazani, Shamsah, Israel, Elliot, Xu, Kai-Feng, Henske, Elizabeth Petri, Blenis, John, Levy, Bruce D, Kwiatkowski, David, Yu, Jane J

“…Lymphangioleiomyomatosis (LAM) is a progressive neoplastic disorder that leads to lung destruction and respiratory failure primarily in women. LAM is typically…”
Get full text

Rapamycin-insensitive up-regulation of adipocyte phospholipase A2 in tuberous sclerosis and lymphangioleiomyomatosis by Li, Chenggang, Zhang, Erik, Sun, Yang, Lee, Po-Shun, Zhan, Yongzhong, Guo, Yanan, Osorio, Juan C, Rosas, Ivan O, Xu, Kai-Feng, Kwiatkowski, David J, Yu, Jane J

“…Tuberous sclerosis syndrome (TSC) is an autosomal dominant tumor suppressor gene syndrome affecting multiple organs, including renal angiomyolipomas and…”
Get full text

Plasma gelsolin depletion and circulating actin in sepsis: a pilot study by Lee, Po-Shun, Patel, Sanjay R, Christiani, David C, Bajwa, Ednan, Stossel, Thomas P, Waxman, Aaron B

“…Depletion of the circulating actin-binding protein, plasma gelsolin (pGSN) has been described in septic patients and animals. We hypothesized that the extent…”
Get full text

Rapamycin-Insensitive Up-Regulation of MMP2 and Other Genes in Tuberous Sclerosis Complex 2-Deficient Lymphangioleiomyomatosis-Like Cells by Lee, Po-Shun, Tsang, Szeman W, Moses, Marsha A, Trayes-Gibson, Zachary, Hsiao, Li-Li, Jensen, Roderick, Squillace, Rachel, Kwiatkowski, David J

“…Increased matrix metalloproteinase (MMP) activity has been implicated in the pathogenesis of lymphangioleiomyomatosis (LAM). The objective of this study was to…”
Get full text

Rapamycin-resistant poly (ADP-ribose) polymerase-1 overexpression is a potential therapeutic target in lymphangioleiomyomatosis by Sun, Yang, Gallacchi, Dana, Zhang, Erik Y, Reynolds, Samuel B, Robinson, Lauren, Malinowska, Izabela A, Chiou, Terry T, Pereira, Ana M, Li, Chenggang, Kwiatkowski, David J, Lee, Po-Shun, Yu, Jane J

“…Lymphangioleiomyomatosis (LAM) is a female-predominant cystic lung disease that can lead to respiratory failure. LAM cells typically have inactivating tuberous…”
Get full text

mTORC1-S6K activation by endotoxin contributes to cytokine up-regulation and early lethality in animals by Lee, Po-Shun, Wilhelmson, Anna S K, Hubner, Anton P, Reynolds, Samuel B, Gallacchi, Dana A, Chiou, Terry T, Kwiatkowski, David J

“…mTORC1 (mammalian target of rapamycin complex 1) activation has been demonstrated in response to endotoxin challenge, but the mechanism and significance are…”
Get full text

Gelsolin and Adipokines Are Associated With Protein-Energy Wasting in Hemodialysis Patients by Chiu, Terry Ting-Yu, Liao, Shang-Chih, Lee, Wen-Chin, Lee, Po-Shun, Ng, Hwee-Yeong, Chien, Yu-Su, Lee, Chien-Te

“…Protein‐energy wasting (PEW) contributes to mortality in hemodialysis (HD) patients. Adipokines regulate energy homeostasis and body weight. Circulating…”
Get full text

Plasma gelsolin is a marker and therapeutic agent in animal sepsis by LEE, Po-Shun, WAXMAN, Aaron B, COTICH, Kara L, SU WOL CHUNG, PERRELLA, Mark A, STOSSEL, Thomas P

Get full text

Oxygen saturation/fraction of inspired oxygen ratio is a simple predictor of noninvasive positive pressure ventilation failure in critically ill patients by Spada, Carol, RRT, Gandhi, Rikesh, Patel, Sanjay R., MD, MS, Nuccio, Paul, MS, RRT, FAARC, Weinhouse, Gerald L., MD, Lee, Po-Shun, MD

“…Abstract Purpose Noninvasive positive pressure ventilation (NPPV) can improve outcomes of critically ill patients. Early and simple predictors of NPPV outcome…”
Get full text

Amplifiers co-translationally enhance CFTR biosynthesis via PCBP1-mediated regulation of CFTR mRNA by Dukovski, Danijela, Villella, Adriana, Bastos, Cecilia, King, Randall, Finley, Daniel, Kelly, Jeffery W., Morimoto, Richard I., Hartl, F. Ulrich, Munoz, Benito, Lee, Po-Shun, Zecevic, Marija, Miller, John Preston

“…•Amplifiers selectively stabilize CFTR mRNA through a co-translational mechanism.•CFTR membrane targeting efficiency is important for amplifier…”
Get full text

Diversity: it is a good thing by Lee, Po-Shun

Get full text

Use of a High-Throughput Phenotypic Screening Strategy to Identify Amplifiers, a Novel Pharmacological Class of Small Molecules That Exhibit Functional Synergy with Potentiators and Correctors by Giuliano, Kenneth A, Wachi, Shinichiro, Drew, Lawrence, Dukovski, Danijela, Green, Olivia, Bastos, Cecilia, Cullen, Matthew D, Hauck, Sheila, Tait, Bradley D, Munoz, Benito, Lee, Po-Shun, Miller, John Preston

“…Cystic fibrosis (CF) is a lethal genetic disorder caused by mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Despite recent…”
Get more information

Plasma Gelsolin and Circulating Actin Correlate with Hemodialysis Mortality by LEE, Po-Shun, SAMPATH, Kartik, THADHANI, Ravi, ANANTH KARUMANCHI, S, TAMEZ, Hector, BHAN, Ishir, ISAKOVA, Tamara, GUTIERREZ, Orlando M, WOLF, Myles, YUCHIAO CHANG, STOSSEL, Thomas P

“…Plasma gelsolin (pGSN) binds actin and bioactive mediators to localize inflammation. Low pGSN correlates with adverse outcomes in acute injury, whereas…”
Get full text

Gelsolin decreases actin toxicity and inflammation in murine multiple sclerosis by Kevin Li-Chun, Hsieh, Schob, Stefan, Zeller, Matthias W.G, Pulli, Benjamin, Ali, Muhammad, Wang, Cuihua, Chiou, Terry Ting-Yu, Tsang, Yuk-Ming, Lee, Po-Shun, Stossel, Thomas P, Chen, John W

“…Abstract Gelsolin is the fourth most abundant protein in the body and its depletion in the blood has been found in multiple sclerosis (MS) patients. How…”
Get full text

Plasma-Type Gelsolin Is Decreased in Human Blood and Cerebrospinal Fluid After Subarachnoid Hemorrhage by CHOU, Sherry H.-Y, LEE, Po-Shun, MINGMING NING, KONIGSBERG, Rachael G, GALLACCI, Dana, CHIOU, Terry, ARAI, Ken, SIMMONS, Suzanne, BAUER, David, FESKE, Steven K, LO, Eng H

“…Subarachnoid hemorrhage (SAH) pathophysiology involves neurovascular proteolysis and inflammation. How these 2 phenomena are related remains unclear. We…”
Get full text

The Effect of Concomitant Sirolimus and Propofol Therapy on Triglyceride Concentrations in Critically Ill Patients by Dube, Kevin M, Szumita, Paul M, Rocchio, Megan A, Lee, Po-Shun, Anger, Kevin E

“…Sirolimus and propofol are both independently associated with the development of hypertriglyceridemia (HTG) during therapy. To date, there are no published…”
Get full text

Relationship of plasma gelsolin levels to outcomes in critically III surgical patients by LEE, Po-Shun, DRAGER, Leslie R, STOSSEL, Thomas P, MOORE, Francis D, ROGERS, Selwyn O

Get full text

The importance of differentiating gelsolin isoforms by Lee, Po-Shun, Waxman, Aaron

Get full text