Article
Misfolding of the cystic fibrosis transmembrane conductance regulator and disease.
Biochemistry - 12 Feb 2008
Cheung Joanne C, Deber Charles M
Abstract excerpt
Understanding the structural basis for defects in protein function that underlie protein-based genetic diseases is the fundamental requirement for development of therapies. This situation is epitomized by the cystic fibrosis transmembrane conductance regulator (CFTR)-the gene product known to be defective in CF patients-that appears particularly susceptible to misfolding when its biogenesis is hampered by...
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