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Nature Medicine, ISSN 1078-8956, 2013, Volume 19, Issue 7, pp. 939 - 945
Journal Article
Nature Cell Biology, ISSN 1465-7392, 09/2010, Volume 12, Issue 9, pp. 863 - 875
Accumulation of unwanted/misfolded proteins in aggregates has been observed in airways of patients with cystic fibrosis (CF), a life-threatening genetic... 
APOPTOSIS | UNFOLDED PROTEIN RESPONSE | BECLIN 1 | DOWN-REGULATION | ENDOPLASMIC-RETICULUM | MICE | GENE-TRANSFER | TISSUE TRANSGLUTAMINASE | NEURODEGENERATIVE DISEASES | CELL-DEATH | CELL BIOLOGY | Reactive Oxygen Species - metabolism | Respiratory Mucosa - drug effects | Sequestosome-1 Protein | Humans | Transglutaminases - genetics | Phosphatidylinositol 3-Kinases - antagonists & inhibitors | Autophagy - drug effects | Young Adult | Cystamine - therapeutic use | Inflammation - metabolism | Autophagy - genetics | Nasal Polyps - metabolism | Mice, Inbred CFTR | Respiratory Mucosa - cytology | Cystic Fibrosis - metabolism | Membrane Proteins - genetics | Mice, Transgenic | Protein Transport - genetics | Apoptosis Regulatory Proteins - metabolism | Reactive Oxygen Species - antagonists & inhibitors | Models, Biological | Cystic Fibrosis - genetics | Transglutaminases - metabolism | Adolescent | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Mice | GTP-Binding Proteins | Cystic Fibrosis - drug therapy | Protein Binding - physiology | Microtubule-Associated Proteins - metabolism | Phosphatidylinositol 3-Kinases - metabolism | Salicylates - pharmacology | Epithelial Sodium Channels - genetics | Apoptosis Regulatory Proteins - genetics | Adult | Cystamine - pharmacology | Membrane Proteins - metabolism | Acetylcysteine - therapeutic use | Beclin-1 | Cystic Fibrosis Transmembrane Conductance Regulator - antagonists & inhibitors | Cell Line | Small Ubiquitin-Related Modifier Proteins - metabolism | Heat-Shock Proteins - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Antioxidants - pharmacology | Mice, Inbred Strains | Nasal Polyps - drug therapy | Antioxidants - therapeutic use | Animals | Acetylcysteine - pharmacology | Adaptor Proteins, Signal Transducing - genetics | Respiratory Mucosa - metabolism | Adaptor Proteins, Signal Transducing - metabolism | Organometallic Compounds - pharmacology | Complications and side effects | Active oxygen | Gene mutations | Lung diseases | Physiological aspects | Cystic fibrosis | Genetic aspects | Research | Health aspects | Risk factors
Journal Article
Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 3/2015, Volume 112, Issue 13, pp. E1614 - E1623
Hypoxic pulmonary vasoconstriction (HPV) optimizes pulmonary ventilation-perfusion matching in regional hypoxia, but promotes pulmonary hypertension in global... 
Ceramide | Transient receptor potential canonical 6 | Pulmonary hypertension | Neutral sphingomyelinase | AIRWAY EPITHELIAL-CELLS | pulmonary hypertension | MULTIDISCIPLINARY SCIENCES | EPOXIDE HYDROLASE | transient receptor potential canonical 6 | SPHINGOSINE 1-PHOSPHATE | TRANSMEMBRANE CONDUCTANCE REGULATOR | neutral sphingomyelinase | ceramide | SMOOTH-MUSCLE | ARTERIAL-HYPERTENSION | LUNG-DISEASE | CYSTIC-FIBROSIS | GAS-EXCHANGE | Calcium - metabolism | Humans | Type C Phospholipases - metabolism | Oxygen - metabolism | Coronary Vessels - metabolism | Pulmonary Artery - metabolism | rho-Associated Kinases - metabolism | Sphingomyelin Phosphodiesterase - metabolism | Lung - metabolism | Receptors, Lysosphingolipid - metabolism | Myocytes, Smooth Muscle - metabolism | Calcium Signaling | Mice, Inbred CFTR | Lung - pathology | TRPC6 Cation Channel | Signal Transduction | Mice, Inbred C57BL | Vasoconstriction | Ceramides - chemistry | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Hypertension, Pulmonary - metabolism | Phosphotransferases (Alcohol Group Acceptor) - metabolism | Protein Transport | Animals | Hypoxia - pathology | TRPC Cation Channels - metabolism | Mice | Heart failure | Care and treatment | Physiological aspects | Muscle cells | Development and progression | Sphingolipids | Research | Biological Sciences | PNAS Plus
Journal Article
Gastroenterology, ISSN 0016-5085, 2011, Volume 141, Issue 4, pp. 1498 - 1508.e5
Background & Aims Loss of function of the cystic fibrosis transmembrane conductance regulator (CFTR) in the biliary epithelium reduces bile flow and... 
Gastroenterology and Hepatology | Biliary Epithelium | Cystic Fibrosis | Src Kinase | TLR4 | LIVER-DISEASE | TRANSMEMBRANE CONDUCTANCE REGULATOR | SIGNAL-TRANSDUCTION | CYTOKINE PRODUCTION | FLUID SECRETION | ENDOTOXIN TOLERANCE | TOLL-LIKE RECEPTORS | NITRIC-OXIDE | CYSTIC-FIBROSIS | SRC FAMILY KINASES | GASTROENTEROLOGY & HEPATOLOGY | Polymyxin B - pharmacology | Dextran Sulfate | Cholagogues and Choleretics - pharmacology | Phosphorylation | Epithelial Cells - metabolism | Leukocyte Common Antigens - metabolism | Ursodeoxycholic Acid - analogs & derivatives | src-Family Kinases | Bile Ducts - metabolism | Colitis - genetics | Epithelial Cells - drug effects | Humans | NF-kappa B - metabolism | Cholangitis - immunology | Transfection | Time Factors | Colitis - chemically induced | HEK293 Cells | Inflammation Mediators - metabolism | Neomycin - pharmacology | Cholangitis - microbiology | Cholangitis - metabolism | Colitis - immunology | Disease Models, Animal | Cholangitis - prevention & control | Mice, Inbred CFTR | Cholangitis - chemically induced | Bile Ducts - drug effects | Cholangitis - genetics | Cytokines - metabolism | Immunity, Innate - drug effects | Mice, Inbred C57BL | Toll-Like Receptor 4 - genetics | Toll-Like Receptor 4 - metabolism | Mice, Knockout | Ursodeoxycholic Acid - pharmacology | Animals | Colitis - microbiology | Epithelial Cells - immunology | Epithelial Cells - microbiology | Colitis - metabolism | Lipopolysaccharides - pharmacology | Anti-Bacterial Agents - pharmacology | Mice | Bile Ducts - microbiology | Keratin-19 - metabolism | Bile Ducts - immunology | inflammatory response | UDCA | signaling | mouse model
Journal Article
Autophagy, ISSN 1554-8627, 11/2014, Volume 10, Issue 11, pp. 2053 - 2074
Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as... 
PM, plasma membrane | autophagy | epigallocatechin gallate | TNF, tumor necrosis factor | CHX, cycloheximide | CXCL2, chemokine (C-X-C motif) ligand 2 | TGM2, transglutaminase 2 | CF, cystic fibrosis | CSNK2, casein kinase 2 | CFTR, cystic fibrosis transmembrane conductance regulator | EGCG, epigallocatechin gallate | SQSTM1, sequestosome 1 | CXCL8, chemokine (C-X-C motif) ligand 8 | cystic fibrosis | cysteamine | sweat chloride | FEV, forced expiratory volume | RPD, rectal potential difference | CFTR | BECN1/Beclin 1, autophagy-related | Sweat chloride | Cysteamine | Cystic fibrosis | Epigallocatechin gallate | Autophagy | GREEN TEA POLYPHENOLS | CELL BIOLOGY | TRANSMEMBRANE CONDUCTANCE REGULATOR | IN-VITRO | GENE | DISEASE | AIRWAY EPITHELIUM | KINASE CK2 | TISSUE TRANSGLUTAMINASE | CHLORIDE | Tumor Necrosis Factor-alpha - metabolism | Sequestosome-1 Protein | Humans | Male | Chlorides - chemistry | Young Adult | Adult | Cystamine - pharmacology | Female | Cell Membrane - metabolism | Membrane Proteins - metabolism | Interleukin-8 - metabolism | Catechin - pharmacology | Child | Cysteamine - administration & dosage | Beclin-1 | Mice, Inbred CFTR | Administration, Oral | Cystic Fibrosis - metabolism | Mice, Transgenic | Apoptosis Regulatory Proteins - metabolism | Homozygote | Phenotype | Pilot Projects | Animals | Adolescent | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Catechin - analogs & derivatives | Mice | Mutation | Adaptor Proteins, Signal Transducing - metabolism
Journal Article
Proceedings of the National Academy of Sciences of the United States of America, ISSN 0027-8424, 8/2014, Volume 111, Issue 34, pp. 12396 - 12401
Journal Article
Gastroenterology, ISSN 0016-5085, 10/2017, Volume 153, Issue 4, pp. 1148 - 1159
BACKGROUND & AIMS: Sjgren's syndrome and autoimmune pancreatitis are disorders with decreased function of salivary, lacrimal glands, and the exocrine pancreas.... 
Duct | Sjögren's syndrome | CFTR | Pancreatitis | Sjogren's syndrome | SJOGRENS-SYNDROME | MODEL | ELECTROLYTE SECRETION | HCO3-SECRETION | FLUID | AUTOIMMUNE PANCREATITIS | CA2 | MUTATION | DISEASE | CYSTIC-FIBROSIS | GASTROENTEROLOGY & HEPATOLOGY | Salivary Glands - immunology | Genetic Therapy | Up-Regulation | Salivary Glands - pathology | Sjogren's Syndrome - immunology | Acinar Cells - immunology | Mice, Inbred MRL lpr | Bone Morphogenetic Protein 6 - genetics | Time Factors | Autoimmune Diseases - metabolism | Autoimmune Diseases - pathology | Salivary Glands - drug effects | Disease Models, Animal | ORAI1 Protein - metabolism | Sjogren's Syndrome - pathology | Transduction, Genetic | Salivation - drug effects | Pancreas - pathology | Autoimmune Diseases - prevention & control | Pancreas - metabolism | Pancreas - immunology | Acinar Cells - pathology | Aquaporin 5 - metabolism | Calcium Signaling - drug effects | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Mice, Inbred NOD | Salivary Glands - metabolism | Quinolones - pharmacology | Pancreatitis - prevention & control | Sjogren's Syndrome - metabolism | Aminophenols - pharmacology | Recovery of Function | Inositol 1,4,5-Trisphosphate Receptors - metabolism | Pancreatitis - immunology | Female | Cyclopropanes - pharmacology | Acinar Cells - metabolism | Chloride Channel Agonists - pharmacology | Tissue Culture Techniques | Autoimmune Diseases - immunology | Pancreas - drug effects | Cystic Fibrosis Transmembrane Conductance Regulator - agonists | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Animals | Bone Morphogenetic Protein 6 - metabolism | Pancreatitis - pathology | Acinar Cells - drug effects | Pancreatitis - metabolism | Sjogren's Syndrome - prevention & control | Cystic fibrosis | Analysis
Journal Article
Nature Medicine, ISSN 1078-8956, 03/2010, Volume 16, Issue 3, pp. 313 - 318
Journal Article
Pflügers Archiv - European Journal of Physiology, ISSN 0031-6768, 6/2015, Volume 467, Issue 6, pp. 1261 - 1275
Journal Article
PLoS ONE, ISSN 1932-6203, 08/2012, Volume 7, Issue 8, p. e44059
Background: Factors determining the onset and severity of chronic obstructive pulmonary disease remain poorly understood. Previous studies demonstrated that... 
TRANSMEMBRANE CONDUCTANCE REGULATOR | CHRONIC-BRONCHITIS | ION-TRANSPORT | EPITHELIAL NA+ CHANNEL | MULTIDISCIPLINARY SCIENCES | MOUSE MODEL | EMPHYSEMA | CYSTIC-FIBROSIS | PULMONARY-DISEASE | EXPRESSION | AIRWAY SURFACE LIQUID | Animals, Newborn | Respiratory System - pathology | Mice, Inbred CFTR | Epithelium - pathology | Lung - pathology | Epithelium - metabolism | Mice, Inbred C57BL | Mice, Transgenic | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Epithelial Sodium Channels - metabolism | Pulmonary Disease, Chronic Obstructive - pathology | Airway Obstruction - metabolism | Disease Progression | Animals | Epithelial Sodium Channels - genetics | Respiratory System - metabolism | Airway Obstruction - pathology | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Lung - metabolism | Mice | Mice, Inbred BALB C | Pulmonary Disease, Chronic Obstructive - metabolism | Pulmonary Disease, Chronic Obstructive - genetics | Neonates | Laboratories | Pathogenesis | Mucus | Dehydration | Respiratory tract | Rodents | Gangrene | Inbreeding | Chronic obstructive pulmonary disease | Bronchitis | Chronic illnesses | Secretion | Mortality | Lung diseases | Test procedures | Ion transport | Cystic fibrosis | Inflammation | Environmental factors | Biophysics | Gene expression | Medical screening | Cigarettes | Studies | Obstructive lung disease | Mutation | Molecular biology | Emphysema
Journal Article
Gastroenterology, ISSN 0016-5085, 2012, Volume 141, Issue 6, pp. 2228 - 2239.e6
Journal Article