John R Riordan
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Explore the profile of John R Riordan including associated specialties, affiliations and a list of published articles.
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67
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3363
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Recent Articles
1.
Sigoillot M, Overtus M, Grodecka M, Scholl D, Garcia-Pino A, Laeremans T, et al.
Nat Commun
. 2019 Jun;
10(1):2636.
PMID: 31201318
The leading cause of cystic fibrosis (CF) is the deletion of phenylalanine 508 (F508del) in the first nucleotide-binding domain (NBD1) of the cystic fibrosis transmembrane conductance regulator (CFTR). The mutation...
2.
Fay J, Aleksandrov L, Jensen T, Cui L, Kousouros J, He L, et al.
Biochemistry
. 2018 Oct;
57(43):6234-6246.
PMID: 30281975
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 with cystic fibrosis, is...
3.
Aleksandrov L, Fay J, Riordan J
Biochemistry
. 2018 Aug;
57(34):5073-5075.
PMID: 30109929
The cystic fibrosis transmembrane conductance regulator (CFTR) is an asymmetric ATP-binding cassette transporter in which ATP hydrolysis occurs only at the second of the two composite nucleotide-binding sites whereas there...
4.
Wang C, Aleksandrov A, Yang Z, Forouhar F, Proctor E, Kota P, et al.
J Biol Chem
. 2018 Jun;
293(46):17685-17704.
PMID: 29903914
Many disease-causing mutations impair protein stability. Here, we explore a thermodynamic strategy to correct the disease-causing F508del mutation in the human cystic fibrosis transmembrane conductance regulator (hCFTR). F508del destabilizes nucleotide-binding...
5.
Yang Z, Hildebrandt E, Jiang F, Aleksandrov A, Khazanov N, Zhou Q, et al.
Biochim Biophys Acta Biomembr
. 2018 Feb;
1860(5):1193-1204.
PMID: 29425673
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is an ABC transporter containing two transmembrane domains forming a chloride ion channel, and two nucleotide binding domains (NBD1 and NBD2). CFTR has...
6.
Ma P, Weichert D, Aleksandrov L, Jensen T, Riordan J, Liu X, et al.
Nat Protoc
. 2017 Aug;
12(9):1745-1762.
PMID: 28771236
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 consequence of instability, it can...
7.
Vernon R, Chong P, Lin H, Yang Z, Zhou Q, Aleksandrov A, et al.
J Biol Chem
. 2017 Jun;
292(34):14147-14164.
PMID: 28655774
Characterization of the second nucleotide-binding domain (NBD2) of the cystic fibrosis transmembrane conductance regulator (CFTR) has lagged behind research into the NBD1 domain, in part because NBD1 contains the F508del...
8.
Das J, Aleksandrov A, Cui L, He L, Riordan J, Dokholyan N
PLoS Comput Biol
. 2017 Jun;
13(6):e1005594.
PMID: 28640808
Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene affect CFTR protein biogenesis or its function as a chloride channel, resulting in dysregulation of epithelial fluid transport in the...
9.
Gakhal A, Jensen T, Bozoky Z, Roldan A, Lukacs G, Forman-Kay J, et al.
MAbs
. 2016 May;
8(6):1167-76.
PMID: 27185291
Cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride channel in the apical surface of epithelial cells in the airway and gastrointestinal tract, and mutation of CFTR is the underlying...
10.
Billet A, Jia Y, Jensen T, Hou Y, Chang X, Riordan J, et al.
Channels (Austin)
. 2015 Dec;
10(3):247-51.
PMID: 26645934
The CFTR chloride channel is tightly regulated by phosphorylation at multiple serine residues. Recently it has been proposed that its activity is also regulated by tyrosine kinases, however the tyrosine...