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Cystic fibrosis: depressed alpha-fucosidase activity in cultured lymphoblasts.
Maler T, Duthie M, Riordan JR. Maler T, et al. FEBS Lett. 1980 Nov 17;121(1):153-6. doi: 10.1016/0014-5793(80)81286-5. FEBS Lett. 1980. PMID: 7461111 No abstract available.
Voltage-dependent block of the cystic fibrosis transmembrane conductance regulator Cl- channel by two closely related arylaminobenzoates.
McCarty NA, McDonough S, Cohen BN, Riordan JR, Davidson N, Lester HA. McCarty NA, et al. J Gen Physiol. 1993 Jul;102(1):1-23. doi: 10.1085/jgp.102.1.1. J Gen Physiol. 1993. PMID: 8397274 Free PMC article.
Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity.
Seibert FS, Linsdell P, Loo TW, Hanrahan JW, Clarke DM, Riordan JR. Seibert FS, et al. J Biol Chem. 1996 Jun 21;271(25):15139-45. doi: 10.1074/jbc.271.25.15139. J Biol Chem. 1996. PMID: 8662892
Crystallographic and single-particle analyses of native- and nucleotide-bound forms of the cystic fibrosis transmembrane conductance regulator (CFTR) protein.
Awayn NH, Rosenberg MF, Kamis AB, Aleksandrov LA, Riordan JR, Ford RC. Awayn NH, et al. Biochem Soc Trans. 2005 Nov;33(Pt 5):996-9. doi: 10.1042/BST20050996. Biochem Soc Trans. 2005. PMID: 16246030
Expression and degradation of the cystic fibrosis transmembrane conductance regulator in Saccharomyces cerevisiae.
Kiser GL, Gentzsch M, Kloser AK, Balzi E, Wolf DH, Goffeau A, Riordan JR. Kiser GL, et al. Arch Biochem Biophys. 2001 Jun 15;390(2):195-205. doi: 10.1006/abbi.2001.2385. Arch Biochem Biophys. 2001. PMID: 11396922
Disease-associated mutations in the extracytoplasmic loops of cystic fibrosis transmembrane conductance regulator do not impede biosynthetic processing but impair chloride channel stability.
Hämmerle MM, Aleksandrov AA, Riordan JR. Hämmerle MM, et al. J Biol Chem. 2001 May 4;276(18):14848-54. doi: 10.1074/jbc.M011017200. Epub 2001 Feb 6. J Biol Chem. 2001. PMID: 11278813
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