메뉴 건너뛰기




Volumn 307, Issue 6, 2014, Pages L431-L434

Rescuing ΔF508 CFTR with trimethylangelicin, A dual-acting corrector and potentiator

Author keywords

Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator; IL 8; Pharmaceutical chaperones

Indexed keywords

4,4',6 TRIMETHYLANGELICIN; CHLORIDE CHANNEL; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DELTAF508 CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; INTERLEUKIN 8; IODIDE; MESSENGER RNA; PROTEIN INHIBITOR; SODIUM CHANNEL; UNCLASSIFIED DRUG; VERTEX 770; VERTEX 809; 4,4',6-TRIMETHYLANGELICIN; CFTR PROTEIN, HUMAN; PSORALEN DERIVATIVE;

EID: 84908498140     PISSN: 10400605     EISSN: 15221504     Source Type: Journal    
DOI: 10.1152/ajplung.00177.2014     Document Type: Article
Times cited : (6)

References (40)
  • 7
    • 0026781952 scopus 로고
    • Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
    • Denning GM, Anderson MP, Amara JF, Marshall J, Smith AE, Welsh MJ. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358: 761-764, 1992.
    • (1992) Nature , vol.358 , pp. 761-764
    • Denning, G.M.1    Anderson, M.P.2    Amara, J.F.3    Marshall, J.4    Smith, A.E.5    Welsh, M.J.6
  • 10
    • 70350236409 scopus 로고    scopus 로고
    • Mechanisms for rescue of correctable folding defects in CFTRA F508
    • Grove DE, Rosser MF, Ren HY, Naren AP, Cyr DM. Mechanisms for rescue of correctable folding defects in CFTRA F508. Mol Biol Cell 20: 4059-4069, 2009.
    • (2009) Mol Biol Cell , vol.20 , pp. 4059-4069
    • Grove, D.E.1    Rosser, M.F.2    Ren, H.Y.3    Naren, A.P.4    Cyr, D.M.5
  • 11
    • 84873436500 scopus 로고    scopus 로고
    • Correctors of AF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein
    • He L, Kota P, Aleksandrov AA, Cui L, Jensen T, Dokholyan NV, Riordan JR. Correctors of AF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein. FASEB J 27: 536-545, 2013.
    • (2013) FASEB J , vol.27 , pp. 536-545
    • He, L.1    Kota, P.2    Aleksandrov, A.A.3    Cui, L.4    Jensen, T.5    Dokholyan, N.V.6    Riordan, J.R.7
  • 12
    • 0035013543 scopus 로고    scopus 로고
    • The AF508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells
    • Heda GD, Tanwani M, Marino CR. The AF508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells. Am J Physiol Cell Physiol 280: C166-C174, 2001.
    • (2001) Am J Physiol Cell Physiol , vol.280 , pp. C166-C174
    • Heda, G.D.1    Tanwani, M.2    Marino, C.R.3
  • 14
    • 84856850146 scopus 로고    scopus 로고
    • Structure-activity relationships of cyanoquino-lines with corrector-potentiator activity in AF508 cystic fibrosis transmembrane conductance regulator protein
    • Knapp JM, Wood AB, Phuan PW, Lodewyk MW, Tantillo DJ, Verkman AS, Kurth MJ. Structure-activity relationships of cyanoquino-lines with corrector-potentiator activity in AF508 cystic fibrosis transmembrane conductance regulator protein. J Med Chem 55: 1242-1251, 2012.
    • (2012) J Med Chem , vol.55 , pp. 1242-1251
    • Knapp, J.M.1    Wood, A.B.2    Phuan, P.W.3    Lodewyk, M.W.4    Tantillo, D.J.5    Verkman, A.S.6    Kurth, M.J.7
  • 18
    • 0027380236 scopus 로고
    • The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells
    • Lukacs GL, Chang XB, Bear C, Kartner N, Mohamed A, Riordan JR, Grinstein S. The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells. J Biol Chem 268: 21592-21598, 1993.
    • (1993) J Biol Chem , vol.268 , pp. 21592-21598
    • Lukacs, G.L.1    Chang, X.B.2    Bear, C.3    Kartner, N.4    Mohamed, A.5    Riordan, J.R.6    Grinstein, S.7
  • 19
    • 0036896008 scopus 로고    scopus 로고
    • Thiazolidinone CFTR inhibitor identified by high-through-put screening blocks cholera toxin-induced intestinal fluid secretion
    • Ma T, Thiagarajah JR, Yang H, Sonawane ND, Folli C, Galietta LJ, Verkman AS. Thiazolidinone CFTR inhibitor identified by high-through-put screening blocks cholera toxin-induced intestinal fluid secretion. J Clin Invest 110: 1651-1658, 2002.
    • (2002) J Clin Invest , vol.110 , pp. 1651-1658
    • Ma, T.1    Thiagarajah, J.R.2    Yang, H.3    Sonawane, N.D.4    Folli, C.5    Galietta, L.J.6    Verkman, A.S.7
  • 21
    • 72049120101 scopus 로고    scopus 로고
    • Design and synthesis of a hybrid potentiator-corrector agonist of the cystic fibrosis mutant protein AF508-CFTR
    • Mills AD, Yoo C, Butler JD, Yang B, Verkman AS, Kurth MJ. Design and synthesis of a hybrid potentiator-corrector agonist of the cystic fibrosis mutant protein AF508-CFTR. Bioorg Med Chem Lett 20: 87-91, 2010.
    • (2010) Bioorg Med Chem Lett , vol.20 , pp. 87-91
    • Mills, A.D.1    Yoo, C.2    Butler, J.D.3    Yang, B.4    Verkman, A.S.5    Kurth, M.J.6
  • 24
    • 24644464284 scopus 로고    scopus 로고
    • Small-molecule correctors of defective AF508-CFTR cellular processing identified by high-throughput screening
    • Pedemonte N, Lukacs GL, Du K, Caci E, Zegarra-Moran O, Galietta LJ, Verkman AS. Small-molecule correctors of defective AF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest 115: 2564-2571, 2005.
    • (2005) J Clin Invest , vol.115 , pp. 2564-2571
    • Pedemonte, N.1    Lukacs, G.L.2    Du, K.3    Caci, E.4    Zegarra-Moran, O.5    Galietta, L.J.6    Verkman, A.S.7
  • 25
    • 79955368563 scopus 로고    scopus 로고
    • Dual activity of amino-arylthiazoles on the trafficking and gating defects of the cystic fibrosis transmembrane conductance regulator chloride channel caused by cystic fibrosis mutations
    • Pedemonte N, Tomati V, Sondo E, Caci E, Millo E, Armirotti A, Damonte G, Zegarra-Moran O, Galietta LJ. Dual activity of amino-arylthiazoles on the trafficking and gating defects of the cystic fibrosis transmembrane conductance regulator chloride channel caused by cystic fibrosis mutations. J Biol Chem 286: 15215-15226, 2011.
    • (2011) J Biol Chem , vol.286 , pp. 15215-15226
    • Pedemonte, N.1    Tomati, V.2    Sondo, E.3    Caci, E.4    Millo, E.5    Armirotti, A.6    Damonte, G.7    Zegarra-Moran, O.8    Galietta, L.J.9
  • 26
    • 80053179441 scopus 로고    scopus 로고
    • Cyanoquinolines with independent corrector and potentiator activities restore APhe508-cystic fibrosis transmembrane conductance regulator chloride channel function in cystic fibrosis
    • Phuan PW, Yang B, Knapp JM, Wood AB, Lukacs GL, Kurth MJ, Verkman AS. Cyanoquinolines with independent corrector and potentiator activities restore APhe508-cystic fibrosis transmembrane conductance regulator chloride channel function in cystic fibrosis. Mol Pharmacol 80: 683-693, 2011.
    • (2011) Mol Pharmacol , vol.80 , pp. 683-693
    • Phuan, P.W.1    Yang, B.2    Knapp, J.M.3    Wood, A.B.4    Lukacs, G.L.5    Kurth, M.J.6    Verkman, A.S.7
  • 28
    • 84884773595 scopus 로고    scopus 로고
    • VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1
    • Ren HY, Grove DE, De La Rosa O, Houck SA, Sopha P, Van Goor F, Hoffman BJ, Cyr DM. VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1. Mol Biol Cell 24: 3016-3024, 2013.
    • (2013) Mol Biol Cell , vol.24 , pp. 3016-3024
    • Ren, H.Y.1    Grove, D.E.2    De La Rosa, O.3    Houck, S.A.4    Sopha, P.5    Van Goor, F.6    Hoffman, B.J.7    Cyr, D.M.8
  • 32
    • 84879000844 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane regulator correctors and potentiators
    • Rowe SM, Verkman AS. Cystic fibrosis transmembrane regulator correctors and potentiators. Cold Spring Harb Perspect Med 3: a015677, 2013.
    • (2013) Cold Spring Harb Perspect Med , vol.3 , pp. a015677
    • Rowe, S.M.1    Verkman, A.S.2
  • 33
    • 0035937847 scopus 로고    scopus 로고
    • Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments
    • Sharma M, Benharouga M, Hu W, Lukacs GL. Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments. J Biol Chem 276: 8942-8950, 2001.
    • (2001) J Biol Chem , vol.276 , pp. 8942-8950
    • Sharma, M.1    Benharouga, M.2    Hu, W.3    Lukacs, G.L.4
  • 38
    • 41149113942 scopus 로고    scopus 로고
    • Enhanced cell-surface stability of rescued AF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones
    • Varga K, Goldstein RF, Jurkuvenaite A, Chen L, Matalon S, Sorscher EJ, Bebok Z, Collawn JF. Enhanced cell-surface stability of rescued AF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones. Biochem J 410: 555-564, 2008.
    • (2008) Biochem J , vol.410 , pp. 555-564
    • Varga, K.1    Goldstein, R.F.2    Jurkuvenaite, A.3    Chen, L.4    Matalon, S.5    Sorscher, E.J.6    Bebok, Z.7    Collawn, J.F.8
  • 39
    • 0028840915 scopus 로고
    • Degradation of CFTR by the ubiquitin-proteasome pathway
    • Ward CL, Omura S, Kopito RR. Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 83: 121-127, 1995.
    • (1995) Cell , vol.83 , pp. 121-127
    • Ward, C.L.1    Omura, S.2    Kopito, R.R.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.