The Overall Structure of Human<unk>  in the Dephosphorylated, ATP-Free Conformation. Domains are labeled. Made from PDB 5UAK The<unk>  gene is approximately 189 kb in length, with 27 exons and 26 introns.<unk>  is a glycoprotein with 1480 amino acids. The protein consists of 7 domains. There are 2<unk>  domains, each with 6 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<unk>  domain by a<unk> y "R" domain that is a unique feature of<unk> , 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 amino-terminus is part of the lasso motif which anchors into the cell membrane. 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.
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