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Science 8 September 1989: Vol. 245. no. 4922, pp. 1066 - 1073 DOI: 10.1126/science.2475911
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Articles
Science, Vol 245, Issue 4922, 1066-1073
Copyright © 1989 by American Association for the Advancement of Science
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
Riordan JR,
JM Rommens,
B Kerem,
N Alon,
R Rozmahel,
Z Grzelczak,
J Zielenski,
S Lok,
N Plavsic,
JL Chou,
and
al. et
Department of Biochemistry, Hospital for Sick Children, Toronto, Ontario, Canada.
Overlapping complementary DNA clones were isolated from epithelial cell libraries with a genomic DNA segment containing a portion of the putative cystic fibrosis (CF) locus, which is on chromosome 7. Transcripts, approximately 6500 nucleotides in size, were detectable in the tissues affected in patients with CF. The predicted protein consists of two similar motifs, each with (i) a domain having properties consistent with membrane association and (ii) a domain believed to be involved in ATP (adenosine triphosphate) binding. A deletion of three base pairs that results in the omission of a phenylalanine residue at the center of the first predicted nucleotide-binding domain was detected in CF patients.
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- Membrane Lateral Diffusion and Capture of CFTR within Transient Confinement Zones.
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- Transmembrane Transport of Endo- and Xenobiotics by Mammalian ATP-Binding Cassette Multidrug Resistance Proteins..
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- The nature of amino acid 482 of human ABCG2 affects substrate transport and ATP hydrolysis but not substrate binding..
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- COX-2: a link between airway inflammation and disordered chloride secretion in cystic fibrosis?.
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- Specific Rescue of Cystic Fibrosis Transmembrane Conductance Regulator Processing Mutants Using Pharmacological Chaperones.
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- The cystic fibrosis transmembrane conductance regulator (Cftr) modulates the timing of puberty in mice..
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- Rescue of {Delta}F508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules.
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- Calreticulin Negatively Regulates the Cell Surface Expression of Cystic Fibrosis Transmembrane Conductance Regulator.
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