Science 8 May 1992: Vol. 256. no. 5058, pp. 774 - 779 DOI: 10.1126/science.1375392

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Articles

Biofilms and type III secretion are not mutually exclusive in Pseudomonas aeruginosa.

H. Mikkelsen, N. J. Bond, M. E. Skindersoe, M. Givskov, K. S. Lilley, and M. Welch (2009)
Microbiology 155, 687-698
 |  Abstract »  |  Full Text »  |  PDF »

Phosphodiesterase 5 Inhibitors and Cystic Fibrosis: Correcting Chloride Channel Dysfunction.

L. L. Clarke (2008)
Am. J. Respir. Crit. Care Med. 177, 469-470
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Cystic Fibrosis Transmembrane Conductance Regulator Activation Is Reduced in the Small Intestine of Na+/H+ Exchanger 3 Regulatory Factor 1 (NHERF-1)- but Not NHERF-2-deficient Mice.

N. Broere, J. Hillesheim, B. Tuo, H. Jorna, A. B. Houtsmuller, S. Shenolikar, E. J. Weinman, M. Donowitz, U. Seidler, H. R. de Jonge, et al. (2007)
J. Biol. Chem. 282, 37575-37584
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Omega-3 polyunsaturated fatty acids improve host response in chronic Pseudomonas aeruginosa lung infection in mice.

M. Pierre, M.-O. Husson, R. L. Berre, J.-L. Desseyn, C. Galabert, L. Beghin, C. Beermann, A. Dagenais, Y. Berthiaume, B. Cardinaud, et al. (2007)
Am J Physiol Lung Cell Mol Physiol 292, L1422-L1431
 |  Abstract »  |  Full Text »  |  PDF »

NHE-RF1 protein rescues {Delta}F508-CFTR function.

F. Bossard, A. Robay, G. Toumaniantz, S. Dahimene, F. Becq, J. Merot, and C. Gauthier (2007)
Am J Physiol Lung Cell Mol Physiol 292, L1085-L1094
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Role of magnesium in the failure of rhDNase therapy in patients with cystic fibrosis..

N N Sanders, H Franckx, K De Boeck, J Haustraete, S C De Smedt, and J Demeester (2006)
Thorax 61, 962-966
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Cystic Fibrosis, Disease Severity, and a Macrophage Migration Inhibitory Factor Polymorphism.

B. J. Plant, C. G. Gallagher, R. Bucala, J. A. Baugh, S. Chappell, L. Morgan, C. M. O'Connor, K. Morgan, and S. C. Donnelly (2005)
Am. J. Respir. Crit. Care Med. 172, 1412-1415
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Expression of the psl Operon in Pseudomonas aeruginosa PAO1 Biofilms: PslA Performs an Essential Function in Biofilm Formation.

J. Overhage, M. Schemionek, J. S. Webb, and B. H. A. Rehm (2005)
Appl. Envir. Microbiol. 71, 4407-4413
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Most F508del-CFTR Is Targeted to Degradation at an Early Folding Checkpoint and Independently of Calnexin.

C. M. Farinha and M. D. Amaral (2005)
Mol. Cell. Biol. 25, 5242-5252
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Transfected {beta}3- but Not {beta}2-Adrenergic Receptors Regulate Cystic Fibrosis Transmembrane Conductance Regulator Activity via a New Pathway Involving the Mitogen-Activated Protein Kinases Extracellular Signal-Regulated Kinases.

A. Robay, G. Toumaniantz, V. Leblais, and C. Gauthier (2005)
Mol. Pharmacol. 67, 648-654
 |  Abstract »  |  Full Text »  |  PDF »

Adult stem cells from bone marrow stroma differentiate into airway epithelial cells: Potential therapy for cystic fibrosis.

G. Wang, B. A. Bunnell, R. G. Painter, B. C. Quiniones, S. Tom, N. A. Lanson Jr, J. L. Spees, D. Bertucci, A. Peister, D. J. Weiss, et al. (2005)
PNAS 102, 186-191
 |  Abstract »  |  Full Text »  |  PDF »

Gene therapy in clinical medicine.

S M Selkirk (2004)
Postgrad. Med. J. 80, 560-570
 |  Abstract »  |  Full Text »  |  PDF »

A domain mimic increases {Delta}F508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia.

L. L. Clarke, L. R. Gawenis, T.-C. Hwang, N. M. Walker, D. B. Gruis, and E. M. Price (2004)
Am J Physiol Cell Physiol 287, C192-C199
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Abnormal Paneth cell granule dissolution and compromised resistance to bacterial colonization in the intestine of CF mice.

L. L. Clarke, L. R. Gawenis, E. M. Bradford, L. M. Judd, K. T. Boyle, J. E. Simpson, G. E. Shull, H. Tanabe, A. J. Ouellette, C. L. Franklin, et al. (2004)
Am J Physiol Gastrointest Liver Physiol 286, G1050-G1058
 |  Abstract »  |  Full Text »  |  PDF »

Detection of Five Common CFTR Mutations by Rapid-Cycle Real-Time Amplification Refractory Mutation System PCR.

E. Dempsey, D. E. Barton, and F. Ryan (2004)
Clin. Chem. 50, 773-775
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CFTR Localization in Native Airway Cells and Cell Lines Expressing Wild-type or F508del-CFTR by a Panel of Different Antibodies.

I. Carvalho-Oliveira, A. Efthymiadou, R. Malho, P. Nogueira, M. Tzetis, E. Kanavakis, M. D. Amaral, and D. Penque (2004)
J. Histochem. Cytochem. 52, 193-203
 |  Abstract »  |  Full Text »  |  PDF »

A Phase I Trial of Intranasal Moli1901 for Cystic Fibrosis.

P. L. Zeitlin, M. P. Boyle, W. B. Guggino, and L. Molina (2004)
Chest 125, 143-149
 |  Abstract »  |  Full Text »  |  PDF »

Mutations in the Nucleotide Binding Domain 1 Signature Motif Region Rescue Processing and Functional Defects of Cystic Fibrosis Transmembrane Conductance Regulator Delta F508.

A. C. V. deCarvalho, L. J. Gansheroff, and J. L. Teem (2002)
J. Biol. Chem. 277, 35896-35905
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis Transmembrane Regulator Gene (CFTR) is Associated with Abnormal Enamel Formation.

C.K. Arquitt, C. Boyd, and J.T. Wright (2002)
Journal of Dental Research 81, 492-496
 |  Abstract »  |  Full Text »  |  PDF »

Long-term Benefits of Inhaled Tobramycin in Adolescent Patients With Cystic Fibrosis.

R. B. Moss (2002)
Chest 121, 55-63
 |  Abstract »  |  Full Text »  |  PDF »

Cystic fibrosis patients with the 3272-26A>G splicing mutation have milder disease than F508del homozygotes: a large European study.

M. D Amaral, P. Pacheco, S. Beck, C. M Farinha, D. Penque, P. Nogueira, C. Barreto, B. Lopes, T. Casals, J. Dapena, et al. (2001)
J. Med. Genet. 38, 777-783
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Factors associated with poor pulmonary function: cross-sectional analysis of data from the ERCF.

J. Navarro, M. Rainisio, H.K. Harms, M.E. Hodson, C. Koch, G. Mastella, B. Strandvik, and S.G. McKenzie (2001)
Eur. Respir. J. 18, 298-305
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Management of Pulmonary Disease in Patients with Cystic Fibrosis.

C. A. Robinson and R. J. Kuhn (2001)
Journal of Pharmacy Practice 14, 207-227
 |  Abstract »  |  PDF »

New Models of the Tracheal Airway Define the Glandular Contribution to Airway Surface Fluid and Electrolyte Composition.

X. Wang, Y. Zhang, A. Amberson, and J. F. Engelhardt (2001)
Am. J. Respir. Cell Mol. Biol. 24, 195-202
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Inflammation and infection in cystic fibrosis - hen or egg?.

N. Hoiby (2001)
Eur. Respir. J. 17, 4-5
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Microbial Biofilms: from Ecology to Molecular Genetics.

M. E. Davey and G. A. O'toole (2000)
Microbiol. Mol. Biol. Rev. 64, 847-867
 |  Abstract »  |  Full Text »  |  PDF »

G1 Phase Growth Arrest and Induction of p21Waf1/Cip1/Sdi1 in IB3-1 Cells Treated with 4-Sodium Phenylbutyrate.

S. A. McGrath-Morrow and J. L. Stahl (2000)
J. Pharmacol. Exp. Ther. 294, 941-947
 |  Abstract »  |  Full Text »

Cystic Fibrosis : End-Stage Care in Canada.

I. Mitchell, E. Nakielna, E. Tullis, and C. Adair (2000)
Chest 118, 80-84
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Innate Lung Defenses and Compromised Pseudomonas aeruginosa Clearance in the Malnourished Mouse Model of Respiratory Infections in Cystic Fibrosis.

H. Yu, S. Z. Nasr, and V. Deretic (2000)
Infect. Immun. 68, 2142-2147
 |  Abstract »  |  Full Text »  |  PDF »

cAMP-dependent Cl- Channel Protein (CFTR) and Its mRNA Are Expressed in the Secretory Portion of Human Eccrine Sweat Gland.

F. Sato and K. Sato (2000)
J. Histochem. Cytochem. 48, 345-354
 |  Abstract »  |  Full Text »

Effects of Methacholine and Uridine 5'-Triphosphate on Tracheal Mucus Rheology in Mice.

E. Sudo, M. M. Lee, W. A. Boyd, and M. King (2000)
Am. J. Respir. Cell Mol. Biol. 22, 373-379
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Shaping a Positive Future for Academic Medicine at Michigan.

G. S. Omenn, A. S. Lichter, L. Warren, and L. C. Bollinger (2000)
JAMA 283, 927-928
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Increasing Epithelial Junction Permeability Enhances Gene Transfer to Airway Epithelia In Vivo.

G. Wang, J. Zabner, C. Deering, J. Launspach, J. Shao, M. Bodner, D. J. Jolly, B. L. Davidson, and P. B. McCray Jr. (2000)
Am. J. Respir. Cell Mol. Biol. 22, 129-138
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Correction of Aberrant Splicing of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene by Antisense Oligonucleotides.

K. J. Friedman, J. Kole, J. A. Cohn, M. R. Knowles, L. M. Silverman, and R. Kole (1999)
J. Biol. Chem. 274, 36193-36199
 |  Abstract »  |  Full Text »  |  PDF »

A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr-/- mice.

S. D. Freedman, M. H. Katz, E. M. Parker, M. Laposata, M. Y. Urman, and J. G. Alvarez (1999)
PNAS 96, 13995-14000
 |  Abstract »  |  Full Text »  |  PDF »

Channelopathies of inwardly rectifying potassium channels.

M. R. ABRAHAM, A. JAHANGIR, A. E. ALEKSEEV, and A. TERZIC (1999)
FASEB J 13, 1901-1910
 |  Abstract »  |  Full Text »

Association of Cystic Fibrosis Transmembrane Conductance Regulator and Protein Phosphatase 2C.

T. Zhu, D. Dahan, A. Evagelidis, S.-X. Zheng, J. Luo, and J. W. Hanrahan (1999)
J. Biol. Chem. 274, 29102-29107
 |  Abstract »  |  Full Text »  |  PDF »

A Six-Month Study of Growth and Energy Expenditure in Children with Cystic Fibrosis Taking a Pulmonary Inhalation Medication (rhDNase).

E. B. Fung, E. M. Barden, D. Wasserman, B. S. Zemel, B. T. Heinrich, T. F. Scanlin, and V. A. Stallings (1999)
J. Am. Coll. Nutr. 18, 330-338
 |  Abstract »  |  Full Text »  |  PDF »

Nucleotide Occlusion in the Human Cystic Fibrosis Transmembrane Conductance Regulator. DIFFERENT PATTERNS IN THE TWO NUCLEOTIDE BINDING DOMAINS.

K. Szabo, G. Szakacs, T. Hegedus, and B. Sarkadi (1999)
J. Biol. Chem. 274, 12209-12212
 |  Abstract »  |  Full Text »  |  PDF »

Desensitization of P2Y2 receptor-activated transepithelial anion secretion.

L. L. Clarke, M. C. Harline, M. A. Otero, G. G. Glover, R. C. Garrad, B. Krugh, N. M. Walker, F. A. Gonzalez, J. T. Turner, and G. A. Weisman (1999)
Am J Physiol Cell Physiol 276, C777-C787
 |  Abstract »  |  Full Text »  |  PDF »

Liver Disease in Children with Cystic Fibrosis: US-Biochemical Comparison in 195 Patients.

H. Patriquin, C. Lenaerts, L. Smith, G. Perreault, A. Grignon, D. Filiatrault, J. Boisvert, C. C. Roy, and A. Rasquin-Weber (1999)
Radiology 211, 229-232
 |  Abstract »  |  Full Text »

Processing of CFTR bearing the P574H mutation differs from wild-type and deltaF508-CFTR.

L. Ostedgaard, B Zeiher, and M. Welsh (1999)
J. Cell Sci. 112, 2091-2098
 |  Abstract »  |  PDF »

Efficient expression of CFTR function with adeno-associated virus vectors that carry shortened CFTR genes.

L. Zhang, D. Wang, H. Fischer, P.-d. Fan, J. H. Widdicombe, Y. W. Kan, and J.-y. Dong (1998)
PNAS 95, 10158-10163
 |  Abstract »  |  Full Text »  |  PDF »

Improved Potency of Hyperactive and Actin-resistant Human DNase I Variants for Treatment of Cystic Fibrosis and Systemic Lupus Erythematosus.

C. Q. Pan, T. H. Dodge, D. L. Baker, W. S. Prince, D. V. Sinicropi, and R. A. Lazarus (1998)
J. Biol. Chem. 273, 18374-18381
 |  Abstract »  |  Full Text »  |  PDF »

Pseudomonas aeruginosa Infection as a Complication of Therapy in Pancreatic Fibrosis (Mucoviscidosis), by Sterling D. Garrard, et al, Pediatrics, 1951;8:482.

B. W. Ramsey (1998)
Pediatrics 102, 210-213
 |  Abstract »  |  Full Text »

Genetics and pulmonary medicine bullet  1: The genetics of cystic fibrosis lung disease.

D. J Davidson and D. J Porteous (1998)
Thorax 53, 389-397
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Research on Cystic Fibrosis . A Journey from the Heart House.

M. J. WELSH and B. W. RAMSEY (1998)
Am. J. Respir. Crit. Care Med. 157, S148-S154
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The Emerging Technology and Application of Preimplantation Genetic Diagnosis.

R. J. Tasca and M. E. McClure (1998)
J. Law Med. Ethics 26, 7-16
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Improved Clearability of Cystic Fibrosis Sputum with Dextran Treatment in vitro.

W. FENG, H. GARRETT, D. P. SPEERT, and M. KING (1998)
Am. J. Respir. Crit. Care Med. 157, 710-714
 |  Abstract »  |  Full Text »  |  PDF »

Microbial Pathogenesis in Cystic Fibrosis: Pulmonary Clearance of Mucoid Pseudomonas aeruginosa and Inflammation in a Mouse Model of Repeated Respiratory Challenge.

H. Yu, M. Hanes, C. E. Chrisp, J. C. Boucher, and V. Deretic (1998)
Infect. Immun. 66, 280-288
 |  Abstract »  |  Full Text »  |  PDF »

RhDNase I Aerosol Deposition and Related Factors in Cystic Fibrosis.

P. DIOT, L. B. PALMER, A. SMALDONE, J. DECELIE-GERMANA, R. GRIMSON, and G. C. SMALDONE (1997)
Am. J. Respir. Crit. Care Med. 156, 1662-1668
 |  Abstract »  |  Full Text »

Genetics of Asthma . Conference Summary.

J. M. DRAZEN and E. K. SILVERMAN (1997)
Am. J. Respir. Crit. Care Med. 156, S69-S71
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Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis.

H. Ishihara and M. J. Welsh (1997)
Am J Physiol Cell Physiol 273, C1278-C1289
 |  Abstract »  |  Full Text »  |  PDF »

Protein phosphatase 2C dephosphorylates and inactivates cystic fibrosis transmembrane conductance regulator.

S. M. Travis, H. A. Berger, and M. J. Welsh (1997)
PNAS 94, 11055-11060
 |  Abstract »  |  Full Text »  |  PDF »

End-of-Life Care in Cystic Fibrosis.

W. M. Robinson, S. Ravilly, C. Berde, and M. E. Wohl (1997)
Pediatrics 100, 205-209
 |  Abstract »  |  Full Text »  |  PDF »

Rheology of Cystic Fibrosis Sputum after in vitro Treatment with Hypertonic Saline Alone and in Combination with Recombinant Human Deoxyribonuclease I.

M. KING, B. DASGUPTA, R. P. TOMKIEWICZ, and N. E. BROWN (1997)
Am. J. Respir. Crit. Care Med. 156, 173-177
 |  Abstract »  |  Full Text »

Cystic Fibrosis Transmembrane Conductance Regulator Inverts Protein Kinase A-mediated Regulation of Epithelial Sodium Channel Single Channel Kinetics.

M. J. Stutts, B. C. Rossier, and R. C. Boucher (1997)
J. Biol. Chem. 272, 14037-14040
 |  Abstract »  |  Full Text »  |  PDF »

Ion Channels -- Basic Science and Clinical Disease.

M. J. Ackerman and D. E. Clapham (1997)
N. Engl. J. Med. 336, 1575-1586
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Benefits and Burdens of Genetic Carrier Identification.

J. K. Williams and D. L. Schutte (1997)
West J Nurs Res 19, 71-81
 |  Abstract »  |  PDF »

Enamel Mineral Composition of Normal and Cystic Fibrosis Transgenic Mice.

J.T. Wright, K.I. Hall, and B.R. Grubb (1996)
Advances in Dental Research 10, 270-275
 |  Abstract »  |  PDF »

Management of Pulmonary Disease in Patients with Cystic Fibrosis.

B. W. Ramsey (1996)
N. Engl. J. Med. 335, 179-188
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Abnormal Enamel Development in a Cystic Fibrosis Transgenic Mouse Model.

J.T. Wright, C.L. Kiefer, K.I. Hall, and B.R. Grubb (1996)
Journal of Dental Research 75, 966-973
 |  Abstract »  |  PDF »

Regulation of Epithelial Sodium Channels by the Cystic Fibrosis Transmembrane Conductance Regulator.

I. I. Ismailov, M. S. Awayda, B. Jovov, B. K. Berdiev, C. M. Fuller, J. R. Dedman, M. A. Kaetzel, and D. J. Benos (1996)
J. Biol. Chem. 271, 4725-4732
 |  Abstract »  |  Full Text »  |  PDF »

Management of the Child With Cystic Fibrosis.

J. H. Tran and K. M. Brennan (1996)
Journal of Pharmacy Practice 9, 75-90
 |  Abstract »  |  PDF »

Isotype-specific Activation of Cystic Fibrosis Transmembrane Conductance Regulator-Chloride Channels by cGMP-dependent Protein Kinase II.

P. J. French, J. Bijman, M. Edixhoven, A. B. Vaandrager, B. J. Scholte, S. M. Lohmann, A. C. Nairn, and H. R. de Jonge (1995)
J. Biol. Chem. 270, 26626-26631
 |  Abstract »  |  Full Text »  |  PDF »

Transfer of Genes to Humans: Early Lessons and Obstacles to Success.

R. G. Crystal (1995)
Science 270, 404-410
 |  Abstract »  |  PDF »

A Controlled Study of Adenoviral-Vector-Mediated Gene Transfer in the Nasal Epithelium of Patients with Cystic Fibrosis.

M. R. Knowles, K. W. Hohneker, Z. Zhou, J. C. Olsen, T. L. Noah, P.-C. Hu, M. W. Leigh, J. F. Engelhardt, L. J. Edwards, K. R. Jones, et al. (1995)
N. Engl. J. Med. 333, 823-831
 |  Abstract »  |  Full Text »  |  PDF »

Alternate Translation Initiation Codons Can Create Functional Forms of Cystic Fibrosis Transmembrane Conductance Regulator.

T. P. Carroll, M. M. Morales, S. B. Fulmer, S. S. Allen, T. R. Flotte, G. R. Cutting, and W. B. Guggino (1995)
J. Biol. Chem. 270, 11941-11946
 |  Abstract »  |  Full Text »  |  PDF »

Management of Sinusitis in Cystic Fibrosis by Endoscopic Surgery and Serial Antimicrobial Lavage: Reduction in Recurrence Requiring Surgery.

R. B. Moss and V. V. King (1995)
Arch Otolaryngol Head Neck Surg 121, 566-572
 |  Abstract »  |  PDF »

cis-Acting Elements Involved in the Regulation of Mouse Clara Cell-specific 10-kDa Protein Gene.

M. K. Ray, S. W. Magdaleno, M. J. Finegold, and F. J. DeMayo (1995)
J. Biol. Chem. 270, 2689-2694
 |  Abstract »  |  Full Text »  |  PDF »

Sequence Comparison of Yeast ATP-binding Cassette Proteins.

S. Michealis and C. Berkower (1995)
Cold Spring Harb Symp Quant Biol 60, 291-307
 |  Abstract »  |  PDF »

A Novel Mutation in the Cystic Fibrosis Gene in Patients with Pulmonary Disease but Normal Sweat Chloride Concentrations.

W. E. Highsmith, L. H. Burch, Z. Zhou, J. C. Olsen, T. E. Boat, A. Spock, J. D. Gorvoy, L. Quittell, K. J. Friedman, L. M. Silverman, et al. (1994)
N. Engl. J. Med. 331, 974-980
 |  Abstract »  |  Full Text »

Effect of Aerosolized Recombinant Human DNase on Exacerbations of Respiratory Symptoms and on Pulmonary Function in Patients with Cystic Fibrosis.

H. J. Fuchs, D. S. Borowitz, D. H. Christiansen, E. M. Morris, M. L. Nash, B. W. Ramsey, B. J. Rosenstein, A. L. Smith, M. E. Wohl, and The Pulmozyme Study Group (1994)
N. Engl. J. Med. 331, 637-642
 |  Abstract »  |  Full Text »

Progress Toward Human Gene Therapy.

M. A. Morsy, K. Mitani, P. Clemens, and C. T. Caskey (1993)
JAMA 270, 2338-2345
 |  Abstract »  |  PDF »

Clinical Physiology of Heritable Photoreceptor Diseases.

A. B. Fulton and M. E. Breton (1993)
Arch Ophthalmol 111, 1479-1481
 |  Abstract »  |  PDF »

Sickle Cell Anemia: {beta}s Gene Cluster Haplotypes as Genetic Markers for Severe Disease Expression.

D. Powars and A. Hiti (1993)
Arch Pediatr Adolesc Med 147, 1197-1202
 |  Abstract »  |  PDF »

Recent Advances in Pulmonary Medicine- First of Two Parts.

S. E. Weinberger (1993)
N. Engl. J. Med. 328, 1389-1397
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SV2, a brain synaptic vesicle protein homologous to bacterial transporters.

S. Bajjalieh, K Peterson, R Shinghal, and R. Scheller (1992)
Science 257, 1271-1273
 |  Abstract »  |  PDF »

An Animal Model for Cystic Fibrosis Made by Gene Targeting.

J. N. Snouwaert, K. K. Brigman, A. M. Latour, N. N. Malouf, R. C. Boucher, O. Smithies, and B. H. Koller (1992)
Science 257, 1083-1088
 |  Abstract »  |  PDF »

Enhanced Activity of a Large Conductance, Calcium-sensitive K+ Channel in the Presence of Src Tyrosine Kinase.

S. Ling, G. Woronuk, L. Sy, S. Lev, and A. P. Braun (2000)
J. Biol. Chem. 275, 30683-30689
 |  Abstract »  |  Full Text »  |  PDF »

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