The CFTR gene is approximately 189 kb in length, with 27 exons and 26 introns.
CFTR is a glycoprotein with 1480 amino acids.
The protein consists of five domains.
There are two transmembrane domains, each with six spans of alpha helices.
These are each connected to a nucleotide binding domain (NBD) in the cytoplasm.
The first NBD is connected to the second transmembrane domain by a regulatory "R" domain that is a unique feature of CFTR, not present in other ABC transporters.
The ion channel only opens when its R-domain has been phosphorylated by PKA and ATP is bound at the NBDs.
The carboxyl terminal of the protein is anchored to the cytoskeleton by a PDZ-interacting domain.
 The structure shown (PDB# 1XMI) shows a homopentameric assembly of mutated NBD1, the first nucleotide binding domain (NBD1) of the transporter.
The CFTR gene is approximately 189 kb in length, with 27 exons and 26 introns. CFTR is a glycoprotein with 1480 amino acids. The protein consists of five domains. There are two transmembrane domains, each with six spans of alpha helices. These are each connected to a nucleotide binding domain (NBD) in the cytoplasm. The first NBD is connected to the second transmembrane domain by a regulatory "R" domain that is a unique feature of CFTR, not present in other ABC transporters. The ion channel only opens when its R-domain has been phosphorylated by PKA and ATP is bound at the NBDs. The carboxyl terminal of the protein is anchored to the cytoskeleton by a PDZ-interacting domain.  The structure shown (PDB# 1XMI) shows a homopentameric assembly of mutated NBD1, the first nucleotide binding domain (NBD1) of the transporter.
Cystic fibrosis transmembrane conductance regulator