[HTML][HTML] Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating

L He, AA Aleksandrov, AWR Serohijos… - Journal of Biological …, 2008 - ASBMB
The cystic fibrosis transmembrane conductance regulator (CFTR) is a unique ATP-binding
cassette (ABC) ion channel mutated in patients with cystic fibrosis. The most common mutation…

Regulatory insertion removal restores maturation, stability and function of ΔF508 CFTR

AA Aleksandrov, P Kota, LA Aleksandrov, L He… - Journal of molecular …, 2010 - Elsevier
The cystic fibrosis transmembrane conductance regulator (CFTR) epithelial anion channel
is a large multidomain membrane protein that matures inefficiently during biosynthesis. Its …

Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains

…, Q Zhou, J An, E Hildebrandt, LA Aleksandrov… - Protein …, 2014 - Wiley Online Library
Detergent interaction with extramembranous soluble domains (ESDs) is not commonly
considered an important determinant of integral membrane protein (IMP) behavior during …

[HTML][HTML] Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR)

MF Rosenberg, AB Kamis, LA Aleksandrov… - Journal of Biological …, 2004 - ASBMB
The cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane protein that
is mutated in patients suffering from cystic fibrosis. Here we report the purification and first …

Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR

AA Aleksandrov, P Kota, L Cui, T Jensen… - Journal of molecular …, 2012 - Elsevier
Most cystic fibrosis is caused by a deletion of a single residue (F508) in CFTR (cystic
fibrosis transmembrane conductance regulator) that disrupts the folding and biosynthetic …

[HTML][HTML] Restoration of domain folding and interdomain assembly by second-site suppressors of the ΔF508 mutation in CFTR

L He, LA Aleksandrov, L Cui, TJ Jensen… - The FASEB …, 2010 - ncbi.nlm.nih.gov
Deletion of PHE508 (ΔF508) from the first nucleotide-binding domain (NBD1) of CFTR, which
causes most cystic fibrosis, disrupts the folding and assembly of the protein. Although the …

Cryo-EM visualization of an active high open probability CFTR anion channel

JF Fay, LA Aleksandrov, TJ Jensen, LL Cui… - Biochemistry, 2018 - ACS Publications
The cystic fibrosis transmembrane conductance regulator (CFTR) anion channel, crucial to
epithelial salt and water homeostasis, and defective due to mutations in its gene in patients …

[HTML][HTML] CFTR (ABCC7) is a hydrolyzable-ligand-gated channel

AA Aleksandrov, LA Aleksandrov… - Pflügers Archiv-European …, 2007 - Springer
As the product of the gene mutated in cystic fibrosis, the most common genetic disease of
Caucasians, CFTR is an atypical ABC protein. From an evolutionary perspective, it is …

[HTML][HTML] The cystic fibrosis transmembrane conductance regulator (CFTR): three-dimensional structure and localization of a channel gate

…, LP O'Ryan, G Hughes, Z Zhao, LA Aleksandrov… - Journal of Biological …, 2011 - ASBMB
Cystic fibrosis affects about 1 in 2500 live births and involves loss of transmembrane chloride
flux due to a lack of a membrane protein channel termed the cystic fibrosis transmembrane …

The cubicon method for concentrating membrane proteins in the cubic mesophase

P Ma, D Weichert, LA Aleksandrov, TJ Jensen… - nature protocols, 2017 - nature.com
The lipid cubic phase (in meso) method is an important approach for generating crystals
and high-resolution X-ray structures of integral membrane proteins. However, as a …