Science 8 September 1989: Vol. 245. no. 4922, pp. 1073 - 1080 DOI: 10.1126/science.2570460

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Articles

Renal Involvement in Cystic Fibrosis: Diseases Spectrum and Clinical Relevance.

Y. Yahiaoui, M. Jablonski, D. Hubert, H. Mosnier-Pudar, L.-H. Noel, M. Stern, D. Grenet, J.-P. Grunfeld, D. Chauveau, and F. Fakhouri (2009)
Clin. J. Am. Soc. Nephrol. 4, 921-928
 |  Abstract »  |  Full Text »  |  PDF »

High-content Functional Screen to Identify Proteins that Correct F508del-CFTR Function.

A. M. Trzcinska-Daneluti, D. Ly, L. Huynh, C. Jiang, C. Fladd, and D. Rotin (2009)
Mol. Cell. Proteomics 8, 780-790
 |  Abstract »  |  Full Text »  |  PDF »

Directed evolution of homing endonuclease I-SceI with altered sequence specificity.

Z. Chen, F. Wen, N. Sun, and H. Zhao (2009)
Protein Eng. Des. Sel. 22, 249-256
 |  Abstract »  |  Full Text »  |  PDF »

Gene copy number variations in Asian patients with congenital bilateral absence of the vas deferens.

C.-H. Lee, C.-C. Wu, Y.-N. Wu, and H.-S. Chiang (2009)
Hum. Reprod. 24, 748-755
 |  Abstract »  |  Full Text »  |  PDF »

Clarification of Laboratory and Clinical Variables That Influence Cystic Fibrosis Newborn Screening With Initial Analysis of Immunoreactive Trypsinogen.

M. Kloosterboer, G. Hoffman, M. Rock, W. Gershan, A. Laxova, Z. Li, and P. M. Farrell (2009)
Pediatrics 123, e338-e346
 |  Abstract »  |  Full Text »  |  PDF »

Genetic basis of HDL variation in 129/SvImJ and C57BL/6J mice: importance of testing candidate genes in targeted mutant mice.

Z. Su, X. Wang, S.-W. Tsaih, A. Zhang, A. Cox, S. Sheehan, and B. Paigen (2009)
J. Lipid Res. 50, 116-125
 |  Abstract »  |  Full Text »  |  PDF »

Can Health-related Quality of Life Predict Survival in Adults with Cystic Fibrosis?.

J. Abbott, A. Hart, A. M. Morton, P. Dey, S. P. Conway, and A. K. Webb (2009)
Am. J. Respir. Crit. Care Med. 179, 54-58
 |  Abstract »  |  Full Text »  |  PDF »

Preventive but Not Late Amiloride Therapy Reduces Morbidity and Mortality of Lung Disease in {beta}ENaC-overexpressing Mice.

Z. Zhou, D. Treis, S. C. Schubert, M. Harm, J. Schatterny, S. Hirtz, J. Duerr, R. C. Boucher, and M. A. Mall (2008)
Am. J. Respir. Crit. Care Med. 178, 1245-1256
 |  Abstract »  |  Full Text »  |  PDF »

Genetic Mapping in Human Disease.

D. Altshuler, M. J. Daly, and E. S. Lander (2008)
Science 322, 881-888
 |  Abstract »  |  Full Text »  |  PDF »

Anemia in Cystic Fibrosis: Incidence, Mechanisms, and Association With Pulmonary Function and Vitamin Deficiency.

A. von Drygalski and J. Biller (2008)
Nutr Clin Pract 23, 557-563
 |  Abstract »  |  Full Text »  |  PDF »

Animal models of chronic lung infection with Pseudomonas aeruginosa: useful tools for cystic fibrosis studies.

I Kukavica-Ibrulj and R C Levesque (2008)
Lab Anim 42, 389-412
 |  Abstract »  |  Full Text »  |  PDF »

Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia.

F. Sun, Z. Mi, S. B. Condliffe, C. A. Bertrand, X. Gong, X. Lu, R. Zhang, J. D. Latoche, J. M. Pilewski, P. D. Robbins, et al. (2008)
FASEB J 22, 3255-3263
 |  Abstract »  |  Full Text »  |  PDF »

Validation of High-Resolution DNA Melting Analysis for Mutation Scanning of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene.

M.-P. Audrezet, A. Dabricot, C. Le Marechal, and C. Ferec (2008)
J. Mol. Diagn. 10, 424-434
 |  Abstract »  |  Full Text »  |  PDF »

A systematic analysis of intronic sequences downstream of 5' splice sites reveals a widespread role for U-rich motifs and TIA1/TIAL1 proteins in alternative splicing regulation.

I. Aznarez, Y. Barash, O. Shai, D. He, J. Zielenski, L.-C. Tsui, J. Parkinson, B. J. Frey, J. M. Rommens, and B. J. Blencowe (2008)
Genome Res. 18, 1247-1258
 |  Abstract »  |  Full Text »  |  PDF »

Nasal Abnormalities in Cystic Fibrosis Mice Independent of Infection and Inflammation.

T. N. Hilliard, J. Zhu, R. Farley, S. Escudero-Garcia, B. J. Wainwright, P. K. Jeffery, U. Griesenbach, A. Bush, J. C. Davies, and E. W. F. W. Alton (2008)
Am. J. Respir. Cell Mol. Biol. 39, 19-25
 |  Abstract »  |  Full Text »  |  PDF »

The Thymidine-Dependent Small-Colony-Variant Phenotype Is Associated with Hypermutability and Antibiotic Resistance in Clinical Staphylococcus aureus Isolates.

S. Besier, J. Zander, B. C. Kahl, P. Kraiczy, V. Brade, and T. A. Wichelhaus (2008)
Antimicrob. Agents Chemother. 52, 2183-2189
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis Transmembrane Conductance Regulator Controls Lung Proteasomal Degradation and Nuclear Factor-{kappa}B Activity in Conditions of Oxidative Stress.

E. Boncoeur, T. Roque, E. Bonvin, V. Saint-Criq, M. Bonora, A. Clement, O. Tabary, A. Henrion-Caude, and J. Jacquot (2008)
Am. J. Pathol. 172, 1184-1194
 |  Abstract »  |  Full Text »  |  PDF »

Development of Chronic Bronchitis and Emphysema in {beta}-Epithelial Na+ Channel-Overexpressing Mice.

M. A. Mall, J. R. Harkema, J. B. Trojanek, D. Treis, A. Livraghi, S. Schubert, Z. Zhou, S. M. Kreda, S. L. Tilley, E. J. Hudson, et al. (2008)
Am. J. Respir. Crit. Care Med. 177, 730-742
 |  Abstract »  |  Full Text »  |  PDF »

Preclinical Evidence that Sildenafil and Vardenafil Activate Chloride Transport in Cystic Fibrosis.

B. Lubamba, H. Lecourt, J. Lebacq, P. Lebecque, H. De Jonge, P. Wallemacq, and T. Leal (2008)
Am. J. Respir. Crit. Care Med. 177, 506-515
 |  Abstract »  |  Full Text »  |  PDF »

Influence of Pancreatic Status and Sex on Polyunsaturated Fatty Acid Profiles in Cystic Fibrosis.

T. C. Coste, G. Deumer, G. Reychler, P. Lebecque, P. Wallemacq, and T. Leal (2008)
Clin. Chem. 54, 388-395
 |  Abstract »  |  Full Text »  |  PDF »

Molecular Proximity of Cystic Fibrosis Transmembrane Conductance Regulator and Epithelial Sodium Channel Assessed by Fluorescence Resonance Energy Transfer.

B. K. Berdiev, E. Cormet-Boyaka, A. Tousson, Y. J. Qadri, H. M. J. Oosterveld-Hut, J. S. Hong, P. A. Gonzales, C. M. Fuller, E. J. Sorscher, G. L. Lukacs, et al. (2007)
J. Biol. Chem. 282, 36481-36488
 |  Abstract »  |  Full Text »  |  PDF »

Yeast gain-of-function mutations reveal structure function relationships conserved among different subfamilies of transient receptor potential channels.

Z. Su, X. Zhou, W. J. Haynes, S. H. Loukin, A. Anishkin, Y. Saimi, and C. Kung (2007)
PNAS 104, 19607-19612
 |  Abstract »  |  Full Text »  |  PDF »

Biogenesis of {gamma}-secretase early in the secretory pathway.

J. Kim, B. Kleizen, R. Choy, G. Thinakaran, S. S. Sisodia, and R. W. Schekman (2007)
J. Cell Biol. 179, 951-963
 |  Abstract »  |  Full Text »  |  PDF »

Adult Cystic Fibrosis.

M. P. Boyle (2007)
JAMA 298, 1787-1793
 |  Abstract »  |  Full Text »  |  PDF »

Protein interactions and disease: computational approaches to uncover the etiology of diseases.

M. G. Kann (2007)
Brief Bioinform 8, 333-346
 |  Abstract »  |  Full Text »  |  PDF »

The Formation of the cAMP/Protein Kinase A-dependent Annexin 2 S100A10 Complex with Cystic Fibrosis Conductance Regulator Protein (CFTR) Regulates CFTR Channel Function.

L. A. Borthwick, J. Mcgaw, G. Conner, C. J. Taylor, V. Gerke, A. Mehta, L. Robson, and R. Muimo (2007)
Mol. Biol. Cell 18, 3388-3397
 |  Abstract »  |  Full Text »  |  PDF »

Patterns of GI disease in adulthood associated with mutations in the CFTR gene.

M. Wilschanski and P. R Durie (2007)
Gut 56, 1153-1163
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Quorum-sensing blockade as a strategy for enhancing host defences against bacterial pathogens.

T. Bjarnsholt and M. Givskov (2007)
Phil Trans R Soc B 362, 1213-1222
 |  Abstract »  |  Full Text »  |  PDF »

A Comparative Study of Five Technologically Diverse CFTR Testing Platforms.

M. A. Johnson, M. J. Yoshitomi, and C. S. Richards (2007)
J. Mol. Diagn. 9, 401-407
 |  Abstract »  |  Full Text »  |  PDF »

Design, Development, Validation, and Use of Synthetic Nucleic Acid Controls for Diagnostic Purposes and Application to Cystic Fibrosis Testing.

T. M. Christensen, M. Jama, V. Ponek, E. Lyon, J. A. Wilson, M. L. Hoffmann, and B. A. Bejjani (2007)
J. Mol. Diagn. 9, 315-319
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis: Lessons from the Sweat Gland.

P. M. Quinton (2007)
Physiology 22, 212-225
 |  Abstract »  |  Full Text »  |  PDF »

Heritability of Lung Disease Severity in Cystic Fibrosis.

L. L. Vanscoy, S. M. Blackman, J. M. Collaco, A. Bowers, T. Lai, K. Naughton, M. Algire, R. McWilliams, S. Beck, J. Hoover-Fong, et al. (2007)
Am. J. Respir. Crit. Care Med. 175, 1036-1043
 |  Abstract »  |  Full Text »  |  PDF »

Prediction of Cellular Immune Responses against CFTR in Patients with Cystic Fibrosis after Gene Therapy.

J. Figueredo, M. P. Limberis, and J. M. Wilson (2007)
Am. J. Respir. Cell Mol. Biol. 36, 529-533
 |  Abstract »  |  Full Text »  |  PDF »

Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin.

Y. Ramu, Y. Xu, and Z. Lu (2007)
PNAS 104, 6448-6453
 |  Abstract »  |  Full Text »  |  PDF »

Protein Kinase CK2, Cystic Fibrosis Transmembrane Conductance Regulator, and the {Delta}F508 Mutation: F508 DELETION DISRUPTS A KINASE-BINDING SITE.

K. J. Treharne, R. M. Crawford, Z. Xu, J.-H. Chen, O. G. Best, E. A. Schulte, D. C. Gruenert, S. M. Wilson, D. N. Sheppard, K. Kunzelmann, et al. (2007)
J. Biol. Chem. 282, 10804-10813
 |  Abstract »  |  Full Text »  |  PDF »

Dysphonia.

D. Seth, S. Chawla, R. Shy, and D. Kamat (2007)
Clinical Pediatrics 46, 283-289
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Single-Nucleotide Polymorphisms and Lung Disease: Clinical Implications.

S. J. Tebbutt, A. James, and P. D. Pare (2007)
Chest 131, 1216-1223
 |  Abstract »  |  Full Text »  |  PDF »

Evaluating candidate agents of selective pressure for cystic fibrosis.

E. M Poolman and A. P Galvani (2007)
J R Soc Interface 4, 91-98
 |  Abstract »  |  Full Text »  |  PDF »

Haplotype Thinking in Lung Disease.

E. K. Silverman (2007)
Proceedings of the ATS 4, 4-8
 |  Abstract »  |  Full Text »  |  PDF »

From the Cover: Recent history of artificial outcrossing facilitates whole-genome association mapping in elite inbred crop varieties.

N. Rostoks, L. Ramsay, K. MacKenzie, L. Cardle, P. R. Bhat, M. L. Roose, J. T. Svensson, N. Stein, R. K. Varshney, D. F. Marshall, et al. (2006)
PNAS 103, 18656-18661
 |  Abstract »  |  Full Text »  |  PDF »

Association Mapping of Complex Trait Loci With Context-Dependent Effects and Unknown Context Variable.

M. J. Sillanpaa and M. Bhattacharjee (2006)
Genetics 174, 1597-1611
 |  Abstract »  |  Full Text »  |  PDF »

GeneRecon--a coalescent based tool for fine-scale association mapping.

T. Mailund, M. H. Schierup, C. N. S. Pedersen, J. N. Madsen, J. Hein, and L. Schauser (2006)
Bioinformatics 22, 2317-2318
 |  Abstract »  |  Full Text »  |  PDF »

De Novo Biosynthetic Profiling of High Abundance Proteins in Cystic Fibrosis Lung Epithelial Cells.

H. B. Pollard, O. Eidelman, C. Jozwik, W. Huang, M. Srivastava, X. D. Ji, B. McGowan, C. F. Norris, T. Todo, T. Darling, et al. (2006)
Mol. Cell. Proteomics 5, 1628-1637
 |  Abstract »  |  Full Text »  |  PDF »

Genetic Dissection of Complex Traits.

E. S. Lander and N. J. Schork (2006)
Focus 4, 442
 |  Abstract »  |  Full Text »  |  PDF »

Regulation of Chemokine Expression by NaCl Occurs Independently of Cystic Fibrosis Transmembrane Conductance Regulator in Macrophages.

A. G. Kostyk, K. M. Dahl, M. W. Wynes, L. A. Whittaker, D. J. Weiss, R. Loi, and D. W.H. Riches (2006)
Am. J. Pathol. 169, 12-20
 |  Abstract »  |  Full Text »  |  PDF »

Polymorphic Markers Suggest a Gene Flow of CFTR Gene from Sub-Saharan/Arabian and Mediterranean to Brazilian Population.

G. MK. Cabello, P. H. Cabello, J. C. Llerena Jr, and O. Fernandes (2006)
J. Hered. 97, 313-317
 |  Abstract »  |  Full Text »  |  PDF »

The cystic fibrosis transmembrane conductance regulator (Cftr) modulates the timing of puberty in mice..

R Jin, C A Hodges, M L Drumm, and M R Palmert (2006)
J. Med. Genet. 43, e29
 |  Abstract »  |  Full Text »  |  PDF »

Abnormalities in the Pulmonary Innate Immune System in Cystic Fibrosis.

T. J. Moraes, J. Plumb, R. Martin, E. Vachon, V. Cherepanov, A. Koh, C. Loeve, J. Jongstra-Bilen, J. H. Zurawska, J. V. Kus, et al. (2006)
Am. J. Respir. Cell Mol. Biol. 34, 364-374
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis Since 1938.

P. B. Davis (2006)
Am. J. Respir. Crit. Care Med. 173, 475-482
 |  Abstract »  |  Full Text »  |  PDF »

A Haplotype Framework for Cystic Fibrosis Mutations in Iran.

E. Elahi, A. Khodadad, I. Kupershmidt, F. Ghasemi, B. Alinasab, R. Naghizadeh, R. G. Eason, M. Amini, M. Esmaili, M. R. Esmaeili Dooki, et al. (2006)
J. Mol. Diagn. 8, 119-127
 |  Abstract »  |  Full Text »  |  PDF »

Detection of an Apparent Homozygous 3120G>A Cystic Fibrosis Mutation on a Routine Carrier Screen.

D. L. Heaney, P. Flume, L. Hamilton, E. Lyon, and D. J Wolff (2006)
J. Mol. Diagn. 8, 137-140
 |  Abstract »  |  Full Text »  |  PDF »

CFTR, chloride concentration and cell volume: could mammalian protein histidine phosphorylation play a latent role?.

K. J Treharne, R. M Crawford, and A Mehta (2006)
Exp Physiol 91, 131-139
 |  Abstract »  |  Full Text »  |  PDF »

Estrogen-Induced Abnormally High Cystic Fibrosis Transmembrane Conductance Regulator Expression Results in Ovarian Hyperstimulation Syndrome.

L. C. Ajonuma, L. L. Tsang, G. H. Zhang, C. H. Y. Wong, M. C. Lau, L. S. Ho, D. K. Rowlands, C. X. Zhou, C. P. Ng, J. Chen, et al. (2005)
Mol. Endocrinol. 19, 3038-3044
 |  Abstract »  |  Full Text »  |  PDF »

Polymorphisms in Cinnamoyl CoA Reductase (CCR) Are Associated With Variation in Microfibril Angle in Eucalyptus spp..

B. R. Thumma, M. F. Nolan, R. Evans, and G. F. Moran (2005)
Genetics 171, 1257-1265
 |  Abstract »  |  Full Text »  |  PDF »

Finding Genetic Modifiers of Cystic Fibrosis.

C. K. Haston and T. J. Hudson (2005)
N. Engl. J. Med. 353, 1509-1511
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CFTR {Delta}F508 mutation has minimal effect on the gene expression profile of differentiated human airway epithelia.

J. Zabner, T. E. Scheetz, H. G. Almabrazi, T. L. Casavant, J. Huang, S. Keshavjee, and P. B. McCray Jr. (2005)
Am J Physiol Lung Cell Mol Physiol 289, L545-L553
 |  Abstract »  |  Full Text »  |  PDF »

The Apical Targeting Signal of the P2Y2 Receptor Is Located in Its First Extracellular Loop.

A.-D. Qi, S. C. Wolff, and R. A. Nicholas (2005)
J. Biol. Chem. 280, 29169-29175
 |  Abstract »  |  Full Text »  |  PDF »

Early Immune Response to the Components of the Type III System of Pseudomonas aeruginosa in Children with Cystic Fibrosis.

R. Corech, A. Rao, A. Laxova, J. Moss, M. J. Rock, Z. Li, M. R. Kosorok, M. L. Splaingard, P. M. Farrell, and J. T. Barbieri (2005)
J. Clin. Microbiol. 43, 3956-3962
 |  Abstract »  |  Full Text »  |  PDF »

Structure and Function of CLCA Proteins.

M. E. Loewen and G. W. Forsyth (2005)
Physiol Rev 85, 1061-1092
 |  Abstract »  |  Full Text »  |  PDF »

Platelet activation in cystic fibrosis.

B. P. O'Sullivan, M. D. Linden, A. L. Frelinger III, M. R. Barnard, M. Spencer-Manzon, J. E. Morris, R. O. Salem, M. Laposata, and A. D. Michelson (2005)
Blood 105, 4635-4641
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis.

S. M. Rowe, S. Miller, and E. J. Sorscher (2005)
N. Engl. J. Med. 352, 1992-2001
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Diagnostic Testing by CFTR Gene Mutation Analysis in a Large Group of Hispanics: Novel Mutations and Assessment of a Population-Specific Mutation Spectrum.

I. Schrijver, S. Ramalingam, R. Sankaran, S. Swanson, C. L.M. Dunlop, S. Keiles, R. B. Moss, J. Oehlert, P. Gardner, E. R. Wassman, et al. (2005)
J. Mol. Diagn. 7, 289-299
 |  Abstract »  |  Full Text »  |  PDF »

The CFTR 3849+10kbC->T and 2789+5G->A alleles are associated with a mild CF phenotype.

I. Dugueperoux and M. De Braekeleer (2005)
Eur. Respir. J. 25, 468-473
 |  Abstract »  |  Full Text »  |  PDF »

Coalescent-Based Association Mapping and Fine Mapping of Complex Trait Loci.

S. Zollner and J. K. Pritchard (2005)
Genetics 169, 1071-1092
 |  Abstract »  |  Full Text »  |  PDF »

Bayesian Association-Based Fine Mapping in Small Chromosomal Segments.

M. J. Sillanpaa and M. Bhattacharjee (2005)
Genetics 169, 427-439
 |  Abstract »  |  Full Text »  |  PDF »

Impact of population structure, effective bottleneck time, and allele frequency on linkage disequilibrium maps.

W. Zhang, A. Collins, J. Gibson, W. J. Tapper, S. Hunt, P. Deloukas, D. R. Bentley, and N. E. Morton (2004)
PNAS 101, 18075-18080
 |  Abstract »  |  Full Text »  |  PDF »

Nutrition for Patients With Cystic Fibrosis.

D. L. Olson and W. F. Schwenk II (2004)
Nutr Clin Pract 19, 575-580
 |  Abstract »  |  Full Text »  |  PDF »

No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis.

A. Werner, M. E. J. Bongers, M. J. Bijvelds, H. R. de Jonge, and H. J. Verkade (2004)
J. Lipid Res. 45, 2277-2286
 |  Abstract »  |  Full Text »  |  PDF »

AlgX Is a Periplasmic Protein Required for Alginate Biosynthesis in Pseudomonas aeruginosa.

A. Robles-Price, T. Y. Wong, H. Sletta, S. Valla, and N. L. Schiller (2004)
J. Bacteriol. 186, 7369-7377
 |  Abstract »  |  Full Text »  |  PDF »

Characterization of cystic fibrosis conductance transmembrane regulator gene mutations and IVS8 poly(T) variants in Portuguese patients with congenital absence of the vas deferens.

A. Grangeia, F. Niel, F. Carvalho, S. Fernandes, A. Ardalan, E. Girodon, J. Silva, L. Ferras, M. Sousa, and A. Barros (2004)
Hum. Reprod. 19, 2502-2508
 |  Abstract »  |  Full Text »  |  PDF »

Mechanism of activation of Xenopus CFTR by stimulation of PKC.

Y. Chen, G. A. Altenberg, and L. Reuss (2004)
Am J Physiol Cell Physiol 287, C1256-C1263
 |  Abstract »  |  Full Text »  |  PDF »

Clinical Manifestations of Cystic Fibrosis Among Patients With Diagnosis in Adulthood.

M. Gilljam, L. Ellis, M. Corey, J. Zielenski, P. Durie, and D. E. Tullis (2004)
Chest 126, 1215-1224
 |  Abstract »  |  Full Text »  |  PDF »

Human chromosome 7 circa 2004: a model for structural and functional studies of the human genome.

S. W. Scherer and E. D. Green (2004)
Hum. Mol. Genet. 13, R303-R313
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis Transmembrane Conductance Regulator Degradation Depends on the Lectins Htm1p/EDEM and the Cdc48 Protein Complex in Yeast.

A. Gnann, J. R. Riordan, and D. H. Wolf (2004)
Mol. Biol. Cell 15, 4125-4135
 |  Abstract »  |  Full Text »  |  PDF »

Activation of Airway Cl- Secretion in Human Subjects by Adenosine.

K. Hentchel-Franks, D. Lozano, V. Eubanks-Tarn, B. Cobb, L. Fan, R. Oster, E. Sorscher, and J. P. Clancy (2004)
Am. J. Respir. Cell Mol. Biol. 31, 140-146
 |  Abstract »  |  Full Text »  |  PDF »

Reversal of cystic fibrosis phenotype in a cultured {Delta}508 cystic fibrosis transmembrane conductance regulator cell line by oligonucleotide insertion.

P. C. Zamecnik, M. K. Raychowdhury, D. R. Tabatadze, and H. F. Cantiello (2004)
PNAS 101, 8150-8155
 |  Abstract »  |  Full Text »  |  PDF »

Digitoxin mimics gene therapy with CFTR and suppresses hypersecretion of IL-8 from cystic fibrosis lung epithelial cells.

M. Srivastava, O. Eidelman, J. Zhang, C. Paweletz, H. Caohuy, Q. Yang, K. A. Jacobson, E. Heldman, W. Huang, C. Jozwik, et al. (2004)
PNAS 101, 7693-7698
 |  Abstract »  |  Full Text »  |  PDF »

Direct Visualization of Cystic Fibrosis Transmembrane Regulator Mutations in the Clinical Laboratory Setting.

C. M. Strom, D. D. Clark, F. M. Hantash, L. Rea, B. Anderson, D. Maul, D. Huang, D. Traul, C. Chen Tubman, R. Garcia, et al. (2004)
Clin. Chem. 50, 836-845
 |  Abstract »  |  Full Text »  |  PDF »

Cardiac Expression of the Cystic Fibrosis Transmembrane Conductance Regulator Involves Novel Exon 1 Usage to Produce a Unique Amino-terminal Protein.

W. L. Davies, J. I. Vandenberg, R. A. Sayeed, and A. E. O. Trezise (2004)
J. Biol. Chem. 279, 15877-15887
 |  Abstract »  |  Full Text »  |  PDF »

Genomics in Sudden Cardiac Death.

D. E. Arking, S. S. Chugh, A. Chakravarti, and P. M. Spooner (2004)
Circ. Res. 94, 712-723
 |  Abstract »  |  Full Text »  |  PDF »

Association between Initial Disease Presentation, Lung Disease Outcomes, and Survival in Patients with Cystic Fibrosis.

H. J. Lai, Y. Cheng, H. Cho, M. R. Kosorok, and P. M. Farrell (2004)
Am. J. Epidemiol. 159, 537-546
 |  Abstract »  |  Full Text »  |  PDF »

{Delta}F508 CFTR Pool in the Endoplasmic Reticulum Is Increased by Calnexin Overexpression.

T. Okiyoneda, K. Harada, M. Takeya, K. Yamahira, I. Wada, T. Shuto, M. A. Suico, Y. Hashimoto, and H. Kai (2004)
Mol. Biol. Cell 15, 563-574
 |  Abstract »  |  Full Text »  |  PDF »

Modulation of Mature Cystic Fibrosis Transmembrane Regulator Protein by the PDZ Domain Protein CAL.

J. Cheng, H. Wang, and W. B. Guggino (2004)
J. Biol. Chem. 279, 1892-1898
 |  Abstract »  |  Full Text »  |  PDF »

Antimicrobial Susceptibility and Synergy Studies of Stenotrophomonas maltophilia Isolates from Patients with Cystic Fibrosis.

P. San Gabriel, J. Zhou, S. Tabibi, Y. Chen, M. Trauzzi, and L. Saiman (2004)
Antimicrob. Agents Chemother. 48, 168-171
 |  Abstract »  |  Full Text »  |  PDF »

New concepts of the pathogenesis of cystic fibrosis lung disease.

R.C. Boucher (2004)
Eur. Respir. J. 23, 146-158
 |  Abstract »  |  Full Text »  |  PDF »

Revisiting Cystic Fibrosis Transmembrane Conductance Regulator Structure and Function.

J. W. Hanrahan and M.-A. Wioland (2004)
Proceedings of the ATS 1, 17-21
 |  Abstract »  |  Full Text »  |  PDF »

Multicenter Characterization and Validation of the Intron-8 Poly(T) Tract (IVS8-T) Status in 25 Coriell Cell Repository Cystic Fibrosis Reference Cell Lines for Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene Mutation Assays.

S. Sebastian, S. G. Spitzer, L. E. Grosso, J. Amos, F. V. Schaefer, E. Lyon, D. J. Wolff, A. Hajianpour, A. K. Taylor, A. Millson, et al. (2004)
Clin. Chem. 50, 251-254
 |  Full Text »  |  PDF »

Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis.

R. L. Gibson, J. L. Burns, and B. W. Ramsey (2003)
Am. J. Respir. Crit. Care Med. 168, 918-951
 |  Abstract »  |  Full Text »  |  PDF »

Mammalian Osmolytes and S-Nitrosoglutathione Promote {Delta}F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein Maturation and Function.

M. Howard, H. Fischer, J. Roux, B. C. Santos, S. R. Gullans, P. H. Yancey, and W. J. Welch (2003)
J. Biol. Chem. 278, 35159-35167
 |  Abstract »  |  Full Text »  |  PDF »

Haplotype Information and Linkage Disequilibrium Mapping for Single Nucleotide Polymorphisms.

X. Lu, T. Niu, and J. S. Liu (2003)
Genome Res. 13, 2112-2117
 |  Abstract »  |  Full Text »  |  PDF »

Characterization of disease-associated mutations affecting an exonic splicing enhancer and two cryptic splice sites in exon 13 of the cystic fibrosis transmembrane conductance regulator gene.

I. Aznarez, E. M. Chan, J. Zielenski, B. J. Blencowe, and L.-C. Tsui (2003)
Hum. Mol. Genet. 12, 2031-2040
 |  Abstract »  |  Full Text »  |  PDF »

Advanced glossary on genetic epidemiology.

N Malats and F Calafell (2003)
J Epidemiol Community Health 57, 562-564
 |  Abstract »  |  Full Text »  |  PDF »

Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas.

N Ahmed, M Corey, G Forstner, J Zielenski, L-C Tsui, L Ellis, E Tullis, and P Durie (2003)
Gut 52, 1159-1164
 |  Abstract »  |  Full Text »  |  PDF »

Efflux and Atherosclerosis: The Clinical and Biochemical Impact of Variations in the ABCA1 Gene.

R. R. Singaraja, L. R. Brunham, H. Visscher, J. J.P. Kastelein, and M. R. Hayden (2003)
Arterioscler. Thromb. Vasc. Biol. 23, 1322-1332
 |  Abstract »  |  Full Text »  |  PDF »

Single Tube Multiplex PCR Detection of 27 Cystic Fibrosis Mutations and 4 Polymorphisms using Neonatal Blood Samples Collected on Guthrie Cards.

G. S Makowski, F. L Nadeau, and S. M Hopfer (2003)
Ann. Clin. Lab. Sci. 33, 243-250
 |  Abstract »  |  Full Text »  |  PDF »

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