Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
about
Epithelial Anion Transport as Modulator of Chemokine SignalingDefective CFTR increases synthesis and mass of sphingolipids that modulate membrane composition and lipid signaling.Reduced GM1 ganglioside in CFTR-deficient human airway cells results in decreased β1-integrin signaling and delayed wound repairNeutral sphingomyelinase 2 deficiency is associated with lung anomalies similar to emphysemaActivation of 3-phosphoinositide-dependent kinase 1 (PDK1) and serum- and glucocorticoid-induced protein kinase 1 (SGK1) by short-chain sphingolipid C4-ceramide rescues the trafficking defect of ΔF508-cystic fibrosis transmembrane conductance regulaPlasma lipidomics reveals potential prognostic signatures within a cohort of cystic fibrosis patients.Acid sphingomyelinase inhibitors normalize pulmonary ceramide and inflammation in cystic fibrosis.Fenretinide corrects the imbalance between omega-6 to omega-3 polyunsaturated fatty acids and inhibits macrophage inflammatory mediators via the ERK pathway.Candidate markers associated with the probability of future pulmonary exacerbations in cystic fibrosis patients.GBA2-encoded β-glucosidase activity is involved in the inflammatory response to Pseudomonas aeruginosa.Replication-Competent Influenza Virus and Respiratory Syncytial Virus Luciferase Reporter Strains Engineered for Co-Infections Identify Antiviral Compounds in Combination Screens.Partial Restoration of CFTR Function in cftr-Null Mice following Targeted Cell Replacement Therapy.The unexpected role of acid sphingomyelinase in cell death and the pathophysiology of common diseases.Defective organellar acidification as a cause of cystic fibrosis lung disease: reexamination of a recurring hypothesis.Revisiting the role of cystic fibrosis transmembrane conductance regulator and counterion permeability in the pH regulation of endocytic organelles.Defective acid sphingomyelinase pathway with Pseudomonas aeruginosa infection in cystic fibrosis.Update in cystic fibrosis 2009.Deregulated balance of omega-6 and omega-3 polyunsaturated fatty acids following infection by the zoonotic pathogen Streptococcus suis.Lipids in cystic fibrosis.Role of phospholipase A2s and lipid mediators in secondary damage after spinal cord injury.Inflammation and its genesis in cystic fibrosis.Repairing the basic defect in cystic fibrosis - one approach is not enough.Clinically Evaluated Cancer Drugs Inhibiting Redox Signaling.Neutrophil Membrane Cholesterol Content is a Key Factor in Cystic Fibrosis Lung Disease.Determinants of culture success in an airway epithelium sampling program of young children with cystic fibrosis.Ceramide in cystic fibrosis: a potential new target for therapeutic intervention.CFTR regulation of intracellular pH and ceramides is required for lung endothelial cell apoptosisIncreased skeletal muscle mitochondrial efficiency in rats with fructose-induced alteration in glucose tolerance.Neutrophil elastase increases airway ceramide levels via upregulation of serine palmitoyltransferase.The protective effect of fenretinide against allergic asthma.Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice.Cystic fibrosis fatty acid imbalance is linked to ceramide deficiency and corrected by fenretinide.Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice.Fenretinide differentially modulates the levels of long- and very long-chain ceramides by downregulating Cers5 enzyme: evidence from bench to bedside.Evidence for the Involvement of Lipid Rafts and Plasma Membrane Sphingolipid Hydrolases in Pseudomonas aeruginosa Infection of Cystic Fibrosis Bronchial Epithelial Cells.Lactosylceramide-accumulation in lipid-rafts mediate aberrant-autophagy, inflammation and apoptosis in cigarette smoke induced emphysema.Cystic fibrosis: the conductance regulator, ceramides, and possible treatments.
P2860
Q26744381-80FBA9BC-B177-4EC0-8238-01BFA6FE980EQ30374361-4C78132C-BCFE-4987-8DA1-0F9A95AC17DDQ33569399-B62977BD-427B-428A-9C7F-9537FC0DCF1CQ33715502-100FBBE5-A7C2-4C17-931D-CD07A74A048CQ34774491-3362D809-AE09-4475-8D0E-0C07EDABE594Q34776256-2E92085E-EF6E-4516-AB5F-277FB2F2C24DQ34994393-D249F078-82C3-47AD-B477-8861E938B4C6Q34999377-3152FA05-B4F7-4558-9034-C401847951A2Q35095433-E7D81A5C-1976-41DF-8B7F-4089C8B4EE81Q35228672-7971E600-5C7B-407D-AAF5-AFFF22E9A873Q35755496-7698FDF6-E966-40DB-9B93-A02522E2E3F0Q36277502-AF3653FC-F662-4DA0-8AC4-3575A853CD99Q36879383-989CFA55-D912-4F7D-A454-73898423E6DBQ37217993-5862BC41-05EC-4685-B030-449C7FE51CB0Q37246334-3B122C36-AD8A-42A8-B8AE-AC8A47322145Q37343771-7C2CAF1B-094E-4B46-BCEF-3324C909E469Q37704053-8AE1F4C1-4B52-4ED2-979D-F3CBA231DAEAQ37713385-51C2640D-DECF-4437-8204-1C4D61E47A56Q37920895-A32CF391-A8A4-4D83-A4B6-F9F457750EEBQ38009737-389E7494-7145-443A-BF8A-A02CCCE991F6Q38579962-77AC4715-EA9B-41E6-8A26-3B2BCDD67F67Q38594795-A4517E21-BB7A-4B75-A5D0-C0FD47C8A253Q38777221-A0B7086A-487D-404F-8452-94194698C32FQ41688386-2D618BD8-A7DC-4DD3-8D71-C18D953A71A3Q41752535-F16FD22D-6877-463E-BA66-C60107B13901Q42005356-02BA9B2C-90E3-4561-B0A0-67D1BA425E30Q42114527-E458E3C8-5273-4B30-BFF6-4B623F211702Q44903535-C287A58A-4CD4-47A6-8326-A567086FE29DQ45069913-A040A015-3E65-481D-B689-0EA3E5187BE9Q45881806-B2E3F39D-65B6-4BC3-A705-D827EAD4434BQ46082134-B0D8D262-8551-474A-8954-E8C5C57ED47FQ46211984-ABD5D41C-D06B-489A-9489-5264B821FA6EQ46320247-9A0A39E5-E8C6-41A4-A71D-12533A5BC2C6Q48148212-B05C98E7-15DE-4743-BC37-4C5390D33E4CQ48247526-A6B8B13F-6BAE-4F72-8CE2-1E5B04DA4403Q51662730-F827A959-2D7E-4FB5-A2BD-EEC7484CFC4EQ52836080-8F14FCAA-7AC8-4A50-96E6-585B98079EEC
P2860
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
description
2008 nî lūn-bûn
@nan
2008 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
name
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@ast
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@en
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@nl
type
label
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@ast
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@en
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@nl
prefLabel
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@ast
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@en
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@nl
P2093
P50
P3181
P356
P1476
Fenretinide corrects newly found ceramide deficiency in cystic fibrosis
@en
P2093
Claude Lachance
Claudine Guilbault
Elias Matouk
Gabriella Wojewodka
Larry C Lands
Thomas A A Skinner
P3181
P356
10.1165/RCMB.2007-0036OC
P407
P577
2007-07-26T00:00:00Z