Article
The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide.
The Journal of biological chemistry - 25 Mar 1992
Thomas P J, Shenbagamurthi P, Sondek J, Hullihen J M, Pedersen P L
Abstract excerpt
Deletion of phenylalanine 508 (delta Phe-508) in the cystic fibrosis transmembrane conductance regulator (CFTR) protein causes approximately 70% of all cases of cystic fibrosis. This residue lies in a region of the protein that we have synthesized chemically and shown to bind adenine nucleotides...
Topics
- Adenosine Triphosphate
- Amino Acid Sequence
- Binding Sites
- Chromosome Deletion
- Circular Dichroism
- Cystic Fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator
- Humans
- Membrane Proteins
- Models, Molecular
- Molecular Sequence Data
- Mutation
- Peptides
- Protein Conformation