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Introduction to section IV: biophysical methods to approach CFTR structure.


ABSTRACT: Inefficient folding of CFTR into a functional three-dimensional structure is the basic pathophysiologic mechanism leading to most cases of cystic fibrosis. Knowledge of the structure of CFTR and placement of these mutations into a structural context would provide information key for developing targeted therapeutic approaches for cystic fibrosis. As a large polytopic membrane protein containing disordered regions, intact CFTR has been refractory to efforts to solve a high-resolution structure using X-ray crystallography. The following chapters summarize current efforts to circumvent these obstacles by utilizing NMR, electron microscopy, and computational methodologies and by development of experimental models of the relevant domains of CFTR.

SUBMITTER: Mendoza JL 

PROVIDER: S-EPMC3498824 | biostudies-literature | 2011

REPOSITORIES: biostudies-literature

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Introduction to section IV: biophysical methods to approach CFTR structure.

Mendoza Juan L JL   Schmidt André A   Thomas Philip J PJ  

Methods in molecular biology (Clifton, N.J.) 20110101


Inefficient folding of CFTR into a functional three-dimensional structure is the basic pathophysiologic mechanism leading to most cases of cystic fibrosis. Knowledge of the structure of CFTR and placement of these mutations into a structural context would provide information key for developing targeted therapeutic approaches for cystic fibrosis. As a large polytopic membrane protein containing disordered regions, intact CFTR has been refractory to efforts to solve a high-resolution structure usi  ...[more]

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