嚢胞性線維症膜コンダクタンス制御因子 (Japanese Wikipedia)

Analysis of information sources in references of the Wikipedia article "嚢胞性線維症膜コンダクタンス制御因子" in Japanese language version.

refsWebsite

Global rank Japanese rank

75nih.gov

4^th place

24^th place

46doi.org

2^nd place

6^th place

10harvard.edu

18^th place

107^th place

2ensembl.org

1,626^th place

2,625^th place

2wikipedia.org

low place

2web.archive.org

1^st place

1univ-montp2.fr

low place

1uq.edu.au

3,558^th place

7,223^rd place

1cftr2.org

low place

1icmr.nic.in

low place

1madsci.org

low place

1cysticfibrosis.org.uk

low place

1sickkids.on.ca

low place

1ucl.ac.uk

1,306^th place

3,177^th place

1cysticfibrosisnewstoday.com

low place

1zenodo.org

621^st place

4,635^th place

1prnewswire.com

406^th place

1,806^th place

1forbes.com

54^th place

269^th place

1nytimes.com

7^th place

63^rd place

cftr2.org

“The Clinical and Functional TRanslation of CFTR (CFTR2): CFTR2 Variant List History”. US CF Foundation, Johns Hopkins University, Cystic Fibrosis Centre at the Hospital for Sick Children in Toronto. 2017年8月2日閲覧。^{[リンク切れ]}

cysticfibrosis.org.uk

ECFS Annual Report: What It Means to the UK Cystic Fibrosis Trust

cysticfibrosisnewstoday.com

“CF Study Finds New Cells Called Ionocytes Carrying High levels of CFTR Gene”. Cystic Fibrosis News Today (2018年8月3日). 2021年5月23日閲覧。

doi.org

“The ABC protein turned chloride channel whose failure causes cystic fibrosis”. Nature 440 (7083): 477–83. (March 2006). Bibcode: 2006Natur.440..477G. doi:10.1038/nature04712. PMC 2720541. PMID 16554808.
“Identification of the cystic fibrosis gene: chromosome walking and jumping”. Science 245 (4922): 1059–65. (September 1989). Bibcode: 1989Sci...245.1059R. doi:10.1126/science.2772657. PMID 2772657.
“Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA”. Science 245 (4922): 1066–73. (September 1989). Bibcode: 1989Sci...245.1066R. doi:10.1126/science.2475911. PMID 2475911.
“Cystic fibrosis transmembrane conductance regulator protein expression in the male excretory duct system during development”. Human Pathology 43 (3): 390–7. (March 2012). doi:10.1016/j.humpath.2011.04.031. PMID 21840567.
“Confirming the phylogeny of mammals by use of large comparative sequence data sets”. Molecular Biology and Evolution 25 (9): 1795–808. (September 2008). doi:10.1093/molbev/msn104. PMC 2515873. PMID 18453548.
“Toward inclusive therapy with CFTR modulators: Progress and challenges”. Pediatric Pulmonology 52 (S48): S4-S14. (November 2017). doi:10.1002/ppul.23773. PMC 6208153. PMID 28881097.
“Cell engineering method using fluorogenic oligonucleotide signaling probes and flow cytometry”. Biotechnology Letters. (March 2021). doi:10.1007/s10529-021-03101-5. PMC 7937778. PMID 33683511.
“Cystic fibrosis”. The New England Journal of Medicine 352 (19): 1992–2001. (May 2005). doi:10.1056/NEJMra043184. PMID 15888700.
“Prevalence of deltaF508, G551D, G542X, and R553X mutations among cystic fibrosis patients in the North of Brazil”. Brazilian Journal of Medical and Biological Research = Revista Brasileira De Pesquisas Medicas E Biologicas 38 (1): 11–5. (January 2005). doi:10.1590/S0100-879X2005000100003. PMID 15665983.
“Cystic fibrosis: a worldwide analysis of CFTR mutations--correlation with incidence data and application to screening”. Human Mutation 19 (6): 575–606. (June 2002). doi:10.1002/humu.10041. PMID 12007216.
“Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease”. American Journal of Physiology. Cell Physiology 284 (1): C2-15. (January 2003). doi:10.1152/ajpcell.00417.2002. PMID 12475759.
“A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein”. The Journal of Biological Chemistry 285 (37): 28741–8. (September 2010). doi:10.1074/jbc.M110.154575. PMC 2937902. PMID 20628052.
“Salmonella typhi uses CFTR to enter intestinal epithelial cells”. Nature 393 (6680): 79–82. (May 1998). Bibcode: 1998Natur.393...79P. doi:10.1038/30006. PMID 9590693.
“Fifteen-year follow-up of pulmonary function in individuals heterozygous for the cystic fibrosis phenylalanine-508 deletion”. The Journal of Allergy and Clinical Immunology 107 (5): 818–23. (May 2001). doi:10.1067/mai.2001.114117. PMID 11344348.
“DeltaF508 heterozygosity in cystic fibrosis and susceptibility to asthma”. Lancet 351 (9120): 1911–3. (June 1998). doi:10.1016/s0140-6736(97)11419-2. PMID 9654257.
“Increased prevalence of chronic rhinosinusitis in carriers of a cystic fibrosis mutation”. Archives of Otolaryngology–Head & Neck Surgery 131 (3): 237–40. (March 2005). doi:10.1001/archotol.131.3.237. PMID 15781764.
“The origin of the major cystic fibrosis mutation (delta F508) in European populations”. Nature Genetics 7 (2): 169–75. (June 1994). doi:10.1038/ng0694-169. PMID 7920636.
“Structure and function of the CFTR chloride channel”. Physiological Reviews 79 (1 Suppl): S23-45. (January 1999). doi:10.1152/physrev.1999.79.1.S23. PMID 9922375.
“An apical PDZ protein anchors the cystic fibrosis transmembrane conductance regulator to the cytoskeleton”. The Journal of Biological Chemistry 273 (31): 19797–801. (July 1998). doi:10.1074/jbc.273.31.19797. PMID 9677412.
“STRUCTURE, GATING, AND REGULATION OF THE CFTR ANION CHANNEL”. Physiological Reviews 99 (1): 707–738. (January 2019). doi:10.1152/physrev.00007.2018. PMID 30516439.
“Epithelial sodium channels (ENaC) are uniformly distributed on motile cilia in the oviduct and the respiratory airways”. Histochemistry and Cell Biology 137 (3): 339–53. (March 2012). doi:10.1007/s00418-011-0904-1. PMID 22207244.
“Localization of epithelial sodium channel (ENaC) and CFTR in the germinal epithelium of the testis, Sertoli cells, and spermatozoa”. Journal of Molecular Histology 49 (2): 195–208. (April 2018). doi:10.1007/s10735-018-9759-2. PMID 29453757.
“Mapping the sites of localization of epithelial sodium channel (ENaC) and CFTR in segments of the mammalian epididymis”. Journal of Molecular Histology 50 (2): 141–154. (April 2019). doi:10.1007/s10735-019-09813-3. PMID 30659401.
“A revised airway epithelial hierarchy includes CFTR-expressing ionocytes”. Nature 560 (7718): 319–324. (August 2018). Bibcode: 2018Natur.560..319M. doi:10.1038/s41586-018-0393-7. PMC 6295155. PMID 30069044.
“A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte”. Nature 560 (7718): 377–381. (August 2018). Bibcode: 2018Natur.560..377P. doi:10.1038/s41586-018-0394-6. PMC 6108322. PMID 30069046.
“Expression of epithelial sodium channel (ENaC) and CFTR in the human epidermis and epidermal appendages”. Histochemistry and Cell Biology 147 (6): 733–748. (June 2017). doi:10.1007/s00418-016-1535-3. PMID 28130590. https://zenodo.org/record/890756.
“The antioxidant role of thiocyanate in the pathogenesis of cystic fibrosis and other inflammation-related diseases”. Proceedings of the National Academy of Sciences of the United States of America 106 (48): 20515–9. (December 2009). Bibcode: 2009PNAS..10620515X. doi:10.1073/pnas.0911412106. PMC 2777967. PMID 19918082.
“Primary hepatocellular carcinoma with severe hypoglycemia: involvement of insulin-like growth factors”. Liver 12 (2): 90–3. (April 1992). doi:10.1111/j.1600-0676.1992.tb00563.x. PMID 1320177.
“Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator”. The Journal of Biological Chemistry 277 (32): 28948–58. (August 2002). doi:10.1074/jbc.M111706200. PMID 12039948.
“A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression”. The Journal of Biological Chemistry 277 (5): 3520–9. (February 2002). doi:10.1074/jbc.M110177200. PMID 11707463.
“The PDZ-binding chloride channel ClC-3B localizes to the Golgi and associates with cystic fibrosis transmembrane conductance regulator-interacting PDZ proteins”. The Journal of Biological Chemistry 278 (8): 6440–9. (February 2003). doi:10.1074/jbc.M211050200. PMID 12471024.
“Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity”. Cell 103 (1): 169–79. (September 2000). doi:10.1016/S0092-8674(00)00096-9. PMID 11051556.
“Protein kinase C epsilon-dependent regulation of cystic fibrosis transmembrane regulator involves binding to a receptor for activated C kinase (RACK1) and RACK1 binding to Na+/H+ exchange regulatory factor”. The Journal of Biological Chemistry 277 (25): 22925–33. (June 2002). doi:10.1074/jbc.M201917200. PMID 11956211.
“The cystic fibrosis transmembrane conductance regulator interacts with and regulates the activity of the HCO3- salvage transporter human Na+-HCO3- cotransport isoform 3”. The Journal of Biological Chemistry 277 (52): 50503–9. (December 2002). doi:10.1074/jbc.M201862200. PMID 12403779.
“CFTR chloride channels are regulated by a SNAP-23/syntaxin 1A complex”. Proceedings of the National Academy of Sciences of the United States of America 99 (19): 12477–82. (September 2002). Bibcode: 2002PNAS...9912477C. doi:10.1073/pnas.192203899. PMC 129470. PMID 12209004.
“C-terminal phosphorylation of MRP2 modulates its interaction with PDZ proteins”. Biochemical and Biophysical Research Communications 302 (3): 454–61. (March 2003). doi:10.1016/S0006-291X(03)00196-7. PMID 12615054.
“Peptide binding consensus of the NHE-RF-PDZ1 domain matches the C-terminal sequence of cystic fibrosis transmembrane conductance regulator (CFTR)”. FEBS Letters 427 (1): 103–8. (May 1998). doi:10.1016/S0014-5793(98)00402-5. PMID 9613608.
“The PDZ-interacting domain of cystic fibrosis transmembrane conductance regulator is required for functional expression in the apical plasma membrane”. The Journal of Biological Chemistry 275 (35): 27069–74. (September 2000). doi:10.1074/jbc.M004951200. PMID 10852925.
“A C-terminal motif found in the beta2-adrenergic receptor, P2Y1 receptor and cystic fibrosis transmembrane conductance regulator determines binding to the Na+/H+ exchanger regulatory factor family of PDZ proteins”. Proceedings of the National Academy of Sciences of the United States of America 95 (15): 8496–501. (July 1998). Bibcode: 1998PNAS...95.8496H. doi:10.1073/pnas.95.15.8496. PMC 21104. PMID 9671706.
“E3KARP mediates the association of ezrin and protein kinase A with the cystic fibrosis transmembrane conductance regulator in airway cells”. The Journal of Biological Chemistry 275 (38): 29539–46. (September 2000). doi:10.1074/jbc.M004961200. PMID 10893422.
“Regulation of CFTR chloride channels by syntaxin and Munc18 isoforms”. Nature 390 (6657): 302–5. (November 1997). Bibcode: 1997Natur.390..302N. doi:10.1038/36882. PMID 9384384.
“Genetics of Cystic Fibrosis: Clinical Implications”. Clinics in Chest Medicine 37 (1): 9–16. (March 2016). doi:10.1016/j.ccm.2015.11.002. PMID 26857764.
“Progress in therapies for cystic fibrosis”. The Lancet. Respiratory Medicine 4 (8): 662–674. (August 2016). doi:10.1016/S2213-2600(16)00023-0. PMID 27053340.
“CFTR inhibitors for treating diarrheal disease”. Clinical Pharmacology and Therapeutics 92 (3): 287–90. (September 2012). doi:10.1038/clpt.2012.114. PMC 3643514. PMID 22850599.
“Emerging treatments in cystic fibrosis”. Drugs 69 (14): 1903–10. (October 2009). doi:10.2165/11318500-000000000-00000. PMID 19747007.
“Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis”. Drugs of Today 49 (4): 253–60. (April 2013). doi:10.1358/dot.2013.49.4.1940984. PMID 23616952.

ensembl.org

May2017.archive.ensembl.org

GRCh38: Ensembl release 89: ENSG00000001626 - Ensembl, May 2017
GRCm38: Ensembl release 89: ENSMUSG00000041301 - Ensembl, May 2017

forbes.com

“The Most Important New Drug Of 2012”. Forbes (2012年12月27日). 2021年5月24日閲覧。

harvard.edu

ui.adsabs.harvard.edu

“The ABC protein turned chloride channel whose failure causes cystic fibrosis”. Nature 440 (7083): 477–83. (March 2006). Bibcode: 2006Natur.440..477G. doi:10.1038/nature04712. PMC 2720541. PMID 16554808.
“Identification of the cystic fibrosis gene: chromosome walking and jumping”. Science 245 (4922): 1059–65. (September 1989). Bibcode: 1989Sci...245.1059R. doi:10.1126/science.2772657. PMID 2772657.
“Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA”. Science 245 (4922): 1066–73. (September 1989). Bibcode: 1989Sci...245.1066R. doi:10.1126/science.2475911. PMID 2475911.
“Salmonella typhi uses CFTR to enter intestinal epithelial cells”. Nature 393 (6680): 79–82. (May 1998). Bibcode: 1998Natur.393...79P. doi:10.1038/30006. PMID 9590693.
“A revised airway epithelial hierarchy includes CFTR-expressing ionocytes”. Nature 560 (7718): 319–324. (August 2018). Bibcode: 2018Natur.560..319M. doi:10.1038/s41586-018-0393-7. PMC 6295155. PMID 30069044.
“A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte”. Nature 560 (7718): 377–381. (August 2018). Bibcode: 2018Natur.560..377P. doi:10.1038/s41586-018-0394-6. PMC 6108322. PMID 30069046.
“The antioxidant role of thiocyanate in the pathogenesis of cystic fibrosis and other inflammation-related diseases”. Proceedings of the National Academy of Sciences of the United States of America 106 (48): 20515–9. (December 2009). Bibcode: 2009PNAS..10620515X. doi:10.1073/pnas.0911412106. PMC 2777967. PMID 19918082.
“CFTR chloride channels are regulated by a SNAP-23/syntaxin 1A complex”. Proceedings of the National Academy of Sciences of the United States of America 99 (19): 12477–82. (September 2002). Bibcode: 2002PNAS...9912477C. doi:10.1073/pnas.192203899. PMC 129470. PMID 12209004.
“A C-terminal motif found in the beta2-adrenergic receptor, P2Y1 receptor and cystic fibrosis transmembrane conductance regulator determines binding to the Na+/H+ exchanger regulatory factor family of PDZ proteins”. Proceedings of the National Academy of Sciences of the United States of America 95 (15): 8496–501. (July 1998). Bibcode: 1998PNAS...95.8496H. doi:10.1073/pnas.95.15.8496. PMC 21104. PMID 9671706.
“Regulation of CFTR chloride channels by syntaxin and Munc18 isoforms”. Nature 390 (6657): 302–5. (November 1997). Bibcode: 1997Natur.390..302N. doi:10.1038/36882. PMID 9384384.

icmr.nic.in

“Association of CFTR gene mutation with bronchial asthma”. The Indian Journal of Medical Research 135 (4): 469–78. (April 2012). PMID 22664493.

madsci.org

“Re: Is there a connection between cystic fibrosis and cholera?”. 2021年5月20日閲覧。

nih.gov

pubmed.ncbi.nlm.nih.gov

“The ABC protein turned chloride channel whose failure causes cystic fibrosis”. Nature 440 (7083): 477–83. (March 2006). Bibcode: 2006Natur.440..477G. doi:10.1038/nature04712. PMC 2720541. PMID 16554808.
“Identification of the cystic fibrosis gene: chromosome walking and jumping”. Science 245 (4922): 1059–65. (September 1989). Bibcode: 1989Sci...245.1059R. doi:10.1126/science.2772657. PMID 2772657.
“Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA”. Science 245 (4922): 1066–73. (September 1989). Bibcode: 1989Sci...245.1066R. doi:10.1126/science.2475911. PMID 2475911.
“Cystic fibrosis transmembrane conductance regulator protein expression in the male excretory duct system during development”. Human Pathology 43 (3): 390–7. (March 2012). doi:10.1016/j.humpath.2011.04.031. PMID 21840567.
“Confirming the phylogeny of mammals by use of large comparative sequence data sets”. Molecular Biology and Evolution 25 (9): 1795–808. (September 2008). doi:10.1093/molbev/msn104. PMC 2515873. PMID 18453548.
“Toward inclusive therapy with CFTR modulators: Progress and challenges”. Pediatric Pulmonology 52 (S48): S4-S14. (November 2017). doi:10.1002/ppul.23773. PMC 6208153. PMID 28881097.
“Cell engineering method using fluorogenic oligonucleotide signaling probes and flow cytometry”. Biotechnology Letters. (March 2021). doi:10.1007/s10529-021-03101-5. PMC 7937778. PMID 33683511.
“Cystic fibrosis”. The New England Journal of Medicine 352 (19): 1992–2001. (May 2005). doi:10.1056/NEJMra043184. PMID 15888700.
“Case studies in cholera: lessons in medical history and science”. The Yale Journal of Biology and Medicine 72 (6): 393–408. (1999). PMC 2579035. PMID 11138935.
“Prevalence of deltaF508, G551D, G542X, and R553X mutations among cystic fibrosis patients in the North of Brazil”. Brazilian Journal of Medical and Biological Research = Revista Brasileira De Pesquisas Medicas E Biologicas 38 (1): 11–5. (January 2005). doi:10.1590/S0100-879X2005000100003. PMID 15665983.
“Cystic fibrosis: a worldwide analysis of CFTR mutations--correlation with incidence data and application to screening”. Human Mutation 19 (6): 575–606. (June 2002). doi:10.1002/humu.10041. PMID 12007216.
“Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease”. American Journal of Physiology. Cell Physiology 284 (1): C2-15. (January 2003). doi:10.1152/ajpcell.00417.2002. PMID 12475759.
“Association of CFTR gene mutation with bronchial asthma”. The Indian Journal of Medical Research 135 (4): 469–78. (April 2012). PMID 22664493.
“A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein”. The Journal of Biological Chemistry 285 (37): 28741–8. (September 2010). doi:10.1074/jbc.M110.154575. PMC 2937902. PMID 20628052.
“Salmonella typhi uses CFTR to enter intestinal epithelial cells”. Nature 393 (6680): 79–82. (May 1998). Bibcode: 1998Natur.393...79P. doi:10.1038/30006. PMID 9590693.
“Fifteen-year follow-up of pulmonary function in individuals heterozygous for the cystic fibrosis phenylalanine-508 deletion”. The Journal of Allergy and Clinical Immunology 107 (5): 818–23. (May 2001). doi:10.1067/mai.2001.114117. PMID 11344348.
“DeltaF508 heterozygosity in cystic fibrosis and susceptibility to asthma”. Lancet 351 (9120): 1911–3. (June 1998). doi:10.1016/s0140-6736(97)11419-2. PMID 9654257.
“Increased prevalence of chronic rhinosinusitis in carriers of a cystic fibrosis mutation”. Archives of Otolaryngology–Head & Neck Surgery 131 (3): 237–40. (March 2005). doi:10.1001/archotol.131.3.237. PMID 15781764.
“The origin of the major cystic fibrosis mutation (delta F508) in European populations”. Nature Genetics 7 (2): 169–75. (June 1994). doi:10.1038/ng0694-169. PMID 7920636.
“Structure and function of the CFTR chloride channel”. Physiological Reviews 79 (1 Suppl): S23-45. (January 1999). doi:10.1152/physrev.1999.79.1.S23. PMID 9922375.
“An apical PDZ protein anchors the cystic fibrosis transmembrane conductance regulator to the cytoskeleton”. The Journal of Biological Chemistry 273 (31): 19797–801. (July 1998). doi:10.1074/jbc.273.31.19797. PMID 9677412.
“STRUCTURE, GATING, AND REGULATION OF THE CFTR ANION CHANNEL”. Physiological Reviews 99 (1): 707–738. (January 2019). doi:10.1152/physrev.00007.2018. PMID 30516439.
“Epithelial sodium channels (ENaC) are uniformly distributed on motile cilia in the oviduct and the respiratory airways”. Histochemistry and Cell Biology 137 (3): 339–53. (March 2012). doi:10.1007/s00418-011-0904-1. PMID 22207244.
“Localization of epithelial sodium channel (ENaC) and CFTR in the germinal epithelium of the testis, Sertoli cells, and spermatozoa”. Journal of Molecular Histology 49 (2): 195–208. (April 2018). doi:10.1007/s10735-018-9759-2. PMID 29453757.
“Mapping the sites of localization of epithelial sodium channel (ENaC) and CFTR in segments of the mammalian epididymis”. Journal of Molecular Histology 50 (2): 141–154. (April 2019). doi:10.1007/s10735-019-09813-3. PMID 30659401.
“A revised airway epithelial hierarchy includes CFTR-expressing ionocytes”. Nature 560 (7718): 319–324. (August 2018). Bibcode: 2018Natur.560..319M. doi:10.1038/s41586-018-0393-7. PMC 6295155. PMID 30069044.
“A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte”. Nature 560 (7718): 377–381. (August 2018). Bibcode: 2018Natur.560..377P. doi:10.1038/s41586-018-0394-6. PMC 6108322. PMID 30069046.
“Expression of epithelial sodium channel (ENaC) and CFTR in the human epidermis and epidermal appendages”. Histochemistry and Cell Biology 147 (6): 733–748. (June 2017). doi:10.1007/s00418-016-1535-3. PMID 28130590. https://zenodo.org/record/890756.
“The antioxidant role of thiocyanate in the pathogenesis of cystic fibrosis and other inflammation-related diseases”. Proceedings of the National Academy of Sciences of the United States of America 106 (48): 20515–9. (December 2009). Bibcode: 2009PNAS..10620515X. doi:10.1073/pnas.0911412106. PMC 2777967. PMID 19918082.
“Primary hepatocellular carcinoma with severe hypoglycemia: involvement of insulin-like growth factors”. Liver 12 (2): 90–3. (April 1992). doi:10.1111/j.1600-0676.1992.tb00563.x. PMID 1320177.
“Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator”. The Journal of Biological Chemistry 277 (32): 28948–58. (August 2002). doi:10.1074/jbc.M111706200. PMID 12039948.
“A Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expression”. The Journal of Biological Chemistry 277 (5): 3520–9. (February 2002). doi:10.1074/jbc.M110177200. PMID 11707463.
“The PDZ-binding chloride channel ClC-3B localizes to the Golgi and associates with cystic fibrosis transmembrane conductance regulator-interacting PDZ proteins”. The Journal of Biological Chemistry 278 (8): 6440–9. (February 2003). doi:10.1074/jbc.M211050200. PMID 12471024.
“Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity”. Cell 103 (1): 169–79. (September 2000). doi:10.1016/S0092-8674(00)00096-9. PMID 11051556.
“Protein kinase C epsilon-dependent regulation of cystic fibrosis transmembrane regulator involves binding to a receptor for activated C kinase (RACK1) and RACK1 binding to Na+/H+ exchange regulatory factor”. The Journal of Biological Chemistry 277 (25): 22925–33. (June 2002). doi:10.1074/jbc.M201917200. PMID 11956211.
“The cystic fibrosis transmembrane conductance regulator interacts with and regulates the activity of the HCO3- salvage transporter human Na+-HCO3- cotransport isoform 3”. The Journal of Biological Chemistry 277 (52): 50503–9. (December 2002). doi:10.1074/jbc.M201862200. PMID 12403779.
“CFTR chloride channels are regulated by a SNAP-23/syntaxin 1A complex”. Proceedings of the National Academy of Sciences of the United States of America 99 (19): 12477–82. (September 2002). Bibcode: 2002PNAS...9912477C. doi:10.1073/pnas.192203899. PMC 129470. PMID 12209004.
“C-terminal phosphorylation of MRP2 modulates its interaction with PDZ proteins”. Biochemical and Biophysical Research Communications 302 (3): 454–61. (March 2003). doi:10.1016/S0006-291X(03)00196-7. PMID 12615054.
“Peptide binding consensus of the NHE-RF-PDZ1 domain matches the C-terminal sequence of cystic fibrosis transmembrane conductance regulator (CFTR)”. FEBS Letters 427 (1): 103–8. (May 1998). doi:10.1016/S0014-5793(98)00402-5. PMID 9613608.
“The PDZ-interacting domain of cystic fibrosis transmembrane conductance regulator is required for functional expression in the apical plasma membrane”. The Journal of Biological Chemistry 275 (35): 27069–74. (September 2000). doi:10.1074/jbc.M004951200. PMID 10852925.
“A C-terminal motif found in the beta2-adrenergic receptor, P2Y1 receptor and cystic fibrosis transmembrane conductance regulator determines binding to the Na+/H+ exchanger regulatory factor family of PDZ proteins”. Proceedings of the National Academy of Sciences of the United States of America 95 (15): 8496–501. (July 1998). Bibcode: 1998PNAS...95.8496H. doi:10.1073/pnas.95.15.8496. PMC 21104. PMID 9671706.
“E3KARP mediates the association of ezrin and protein kinase A with the cystic fibrosis transmembrane conductance regulator in airway cells”. The Journal of Biological Chemistry 275 (38): 29539–46. (September 2000). doi:10.1074/jbc.M004961200. PMID 10893422.
“Regulation of CFTR chloride channels by syntaxin and Munc18 isoforms”. Nature 390 (6657): 302–5. (November 1997). Bibcode: 1997Natur.390..302N. doi:10.1038/36882. PMID 9384384.
“Genetics of Cystic Fibrosis: Clinical Implications”. Clinics in Chest Medicine 37 (1): 9–16. (March 2016). doi:10.1016/j.ccm.2015.11.002. PMID 26857764.
“Progress in therapies for cystic fibrosis”. The Lancet. Respiratory Medicine 4 (8): 662–674. (August 2016). doi:10.1016/S2213-2600(16)00023-0. PMID 27053340.
“CFTR inhibitors for treating diarrheal disease”. Clinical Pharmacology and Therapeutics 92 (3): 287–90. (September 2012). doi:10.1038/clpt.2012.114. PMC 3643514. PMID 22850599.
“Emerging treatments in cystic fibrosis”. Drugs 69 (14): 1903–10. (October 2009). doi:10.2165/11318500-000000000-00000. PMID 19747007.
“Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis”. Drugs of Today 49 (4): 253–60. (April 2013). doi:10.1358/dot.2013.49.4.1940984. PMID 23616952.

ncbi.nlm.nih.gov

pmc.ncbi.nlm.nih.gov

nytimes.com

“The $300,000 Drug”. New York Times (2014年7月18日). 2021年5月24日閲覧。

prnewswire.com

“Phase 3 Study of VX-770 Shows Marked Improvement in Lung Function Among People with Cystic Fibrosis with G551D Mutation”. Press Release. Cystic Fibrosis Foundation (2011年2月23日). 2021年5月24日閲覧。

sickkids.on.ca

genet.sickkids.on.ca

Cystic Fibrosis Mutation Database. “Genomic DNA sequence”. 2016年8月22日時点のオリジナルよりアーカイブ。2013年4月6日閲覧。

ucl.ac.uk

discovery.ucl.ac.uk

“STRUCTURE, GATING, AND REGULATION OF THE CFTR ANION CHANNEL”. Physiological Reviews 99 (1): 707–738. (January 2019). doi:10.1152/physrev.00007.2018. PMID 30516439.

univ-montp2.fr

orthomam.univ-montp2.fr

“OrthoMaM phylogenetic marker: CFTR coding sequence”. 2016年3月2日時点のオリジナルよりアーカイブ。2010年3月12日閲覧。

uq.edu.au

espace.library.uq.edu.au

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