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Arrest of CFTRΔF508 folding
被引:21
作者
:
Cyr, DM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
Cyr, DM
[
1
]
机构
:
[1]
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
[2]
Univ N Carolina, N Carolina Cyst Fibrosis Res Ctr, Chapel Hill, NC 27599 USA
来源
:
NATURE STRUCTURAL & MOLECULAR BIOLOGY
|
2005年
/ 12卷
/ 01期
关键词
:
D O I
:
10.1038/nsmb0105-2
中图分类号
:
Q5 [生物化学];
Q7 [分子生物学];
学科分类号
:
071010 ;
081704 ;
摘要
:
The deletion of residue 508 in CFTR is the most common cystic fibrosis-causing mutation. Recent studies indicate that the main chain and side chain of this residue contribute to the proper folding of CFTR at different stages.
引用
收藏
页码:2 / 3
页数:2
相关论文
共 18 条
[1]
RETRACTED: Structure of MsbA from E-coli:: A homolog of the multidrug resistance ATP binding cassette (ABC) transporters (Retracted Article. See vol 314, pg 1875, 2006)
Chang, G
论文数:
0
引用数:
0
h-index:
0
机构:
Scripps Res Inst, Dept Mol Biol, La Jolla, CA 92037 USA
Scripps Res Inst, Dept Mol Biol, La Jolla, CA 92037 USA
Chang, G
Roth, CB
论文数:
0
引用数:
0
h-index:
0
机构:
Scripps Res Inst, Dept Mol Biol, La Jolla, CA 92037 USA
Scripps Res Inst, Dept Mol Biol, La Jolla, CA 92037 USA
Roth, CB
[J].
SCIENCE,
2001,
293
(5536)
: 1793
-
1800
[2]
The ΔF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator
Chen, EY
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Dept Med, Canadian Inst Hlth Res Grp Membrane Biol, Toronto, ON M5S 1A8, Canada
Chen, EY
Bartlett, MC
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Dept Med, Canadian Inst Hlth Res Grp Membrane Biol, Toronto, ON M5S 1A8, Canada
Bartlett, MC
Loo, TW
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Dept Med, Canadian Inst Hlth Res Grp Membrane Biol, Toronto, ON M5S 1A8, Canada
Loo, TW
Clarke, DM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Dept Med, Canadian Inst Hlth Res Grp Membrane Biol, Toronto, ON M5S 1A8, Canada
Clarke, DM
[J].
JOURNAL OF BIOLOGICAL CHEMISTRY,
2004,
279
(38)
: 39620
-
39627
[3]
Protein quality control:: U-box-containing E3 ubiquitin ligases join the fold
Cyr, DM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
Cyr, DM
Höhfeld, J
论文数:
0
引用数:
0
h-index:
0
机构:
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
Höhfeld, J
Patterson, C
论文数:
0
引用数:
0
h-index:
0
机构:
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
Patterson, C
[J].
TRENDS IN BIOCHEMICAL SCIENCES,
2002,
27
(07)
: 368
-
375
[4]
Protein folding and misfolding
Dobson, CM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Cambridge, Dept Chem, Cambridge CB2 1EW, England
Univ Cambridge, Dept Chem, Cambridge CB2 1EW, England
Dobson, CM
[J].
NATURE,
2003,
426
(6968)
: 884
-
890
[5]
The F508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR
Du, K
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Hosp Sick Children, Res Inst, Program Cell & Lung Biol, Toronto, ON M5G 1X8, Canada
Du, K
Sharma, M
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Hosp Sick Children, Res Inst, Program Cell & Lung Biol, Toronto, ON M5G 1X8, Canada
Sharma, M
Lukacs, GL
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Hosp Sick Children, Res Inst, Program Cell & Lung Biol, Toronto, ON M5G 1X8, Canada
Univ Toronto, Hosp Sick Children, Res Inst, Program Cell & Lung Biol, Toronto, ON M5G 1X8, Canada
Lukacs, GL
[J].
NATURE STRUCTURAL & MOLECULAR BIOLOGY,
2005,
12
(01)
: 17
-
25
[6]
Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis
Gelman, MS
论文数:
0
引用数:
0
h-index:
0
机构:
Stanford Univ, Dept Biol Sci, Stanford, CA 94305 USA
Gelman, MS
Kopito, RR
论文数:
0
引用数:
0
h-index:
0
机构:
Stanford Univ, Dept Biol Sci, Stanford, CA 94305 USA
Stanford Univ, Dept Biol Sci, Stanford, CA 94305 USA
Kopito, RR
[J].
JOURNAL OF CLINICAL INVESTIGATION,
2002,
110
(11)
: 1591
-
1597
[7]
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
Lewis, HA
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Lewis, HA
Buchanan, SG
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Buchanan, SG
Burley, SK
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Burley, SK
Conners, K
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Conners, K
Dickey, M
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Dickey, M
Dorwart, M
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Dorwart, M
Fowler, R
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Fowler, R
Gao, X
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Gao, X
Guggino, WB
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Guggino, WB
Hendrickson, WA
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Hendrickson, WA
Hunt, JF
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Hunt, JF
Kearins, MC
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Kearins, MC
Lorimer, D
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Lorimer, D
Maloney, PC
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Maloney, PC
Post, KW
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Post, KW
Rajashankar, KR
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Rajashankar, KR
Rutter, ME
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Rutter, ME
Sauder, JM
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Sauder, JM
Shriver, S
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Shriver, S
Thibodeau, PH
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Thibodeau, PH
Thomas, PJ
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Thomas, PJ
Zhang, M
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Zhang, M
Zhao, X
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Zhao, X
Emtage, S
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Emtage, S
[J].
EMBO JOURNAL,
2004,
23
(02)
: 282
-
293
[8]
LEWIS HA, 2004, J BIOL CHEM 1103
[9]
The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis
Meacham, GC
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Meacham, GC
Lu, Z
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Lu, Z
King, S
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
King, S
Sorscher, E
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Sorscher, E
Tousson, A
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Tousson, A
Cyr, DM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Cyr, DM
[J].
EMBO JOURNAL,
1999,
18
(06)
: 1492
-
1505
[10]
The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation
Meacham, GC
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama Birmingham, Med Ctr, Dept Cell Biol, Birmingham, AL 35294 USA
Meacham, GC
Patterson, C
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama Birmingham, Med Ctr, Dept Cell Biol, Birmingham, AL 35294 USA
Patterson, C
Zhang, WY
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama Birmingham, Med Ctr, Dept Cell Biol, Birmingham, AL 35294 USA
Zhang, WY
Younger, JM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama Birmingham, Med Ctr, Dept Cell Biol, Birmingham, AL 35294 USA
Younger, JM
Cyr, DM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama Birmingham, Med Ctr, Dept Cell Biol, Birmingham, AL 35294 USA
Cyr, DM
[J].
NATURE CELL BIOLOGY,
2001,
3
(01)
: 100
-
105
←
1
2
→
共 18 条
[1]
RETRACTED: Structure of MsbA from E-coli:: A homolog of the multidrug resistance ATP binding cassette (ABC) transporters (Retracted Article. See vol 314, pg 1875, 2006)
Chang, G
论文数:
0
引用数:
0
h-index:
0
机构:
Scripps Res Inst, Dept Mol Biol, La Jolla, CA 92037 USA
Scripps Res Inst, Dept Mol Biol, La Jolla, CA 92037 USA
Chang, G
Roth, CB
论文数:
0
引用数:
0
h-index:
0
机构:
Scripps Res Inst, Dept Mol Biol, La Jolla, CA 92037 USA
Scripps Res Inst, Dept Mol Biol, La Jolla, CA 92037 USA
Roth, CB
[J].
SCIENCE,
2001,
293
(5536)
: 1793
-
1800
[2]
The ΔF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator
Chen, EY
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Dept Med, Canadian Inst Hlth Res Grp Membrane Biol, Toronto, ON M5S 1A8, Canada
Chen, EY
Bartlett, MC
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Dept Med, Canadian Inst Hlth Res Grp Membrane Biol, Toronto, ON M5S 1A8, Canada
Bartlett, MC
Loo, TW
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Dept Med, Canadian Inst Hlth Res Grp Membrane Biol, Toronto, ON M5S 1A8, Canada
Loo, TW
Clarke, DM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Dept Med, Canadian Inst Hlth Res Grp Membrane Biol, Toronto, ON M5S 1A8, Canada
Clarke, DM
[J].
JOURNAL OF BIOLOGICAL CHEMISTRY,
2004,
279
(38)
: 39620
-
39627
[3]
Protein quality control:: U-box-containing E3 ubiquitin ligases join the fold
Cyr, DM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
Cyr, DM
Höhfeld, J
论文数:
0
引用数:
0
h-index:
0
机构:
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
Höhfeld, J
Patterson, C
论文数:
0
引用数:
0
h-index:
0
机构:
Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA
Patterson, C
[J].
TRENDS IN BIOCHEMICAL SCIENCES,
2002,
27
(07)
: 368
-
375
[4]
Protein folding and misfolding
Dobson, CM
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Cambridge, Dept Chem, Cambridge CB2 1EW, England
Univ Cambridge, Dept Chem, Cambridge CB2 1EW, England
Dobson, CM
[J].
NATURE,
2003,
426
(6968)
: 884
-
890
[5]
The F508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR
Du, K
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Hosp Sick Children, Res Inst, Program Cell & Lung Biol, Toronto, ON M5G 1X8, Canada
Du, K
Sharma, M
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Hosp Sick Children, Res Inst, Program Cell & Lung Biol, Toronto, ON M5G 1X8, Canada
Sharma, M
Lukacs, GL
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Toronto, Hosp Sick Children, Res Inst, Program Cell & Lung Biol, Toronto, ON M5G 1X8, Canada
Univ Toronto, Hosp Sick Children, Res Inst, Program Cell & Lung Biol, Toronto, ON M5G 1X8, Canada
Lukacs, GL
[J].
NATURE STRUCTURAL & MOLECULAR BIOLOGY,
2005,
12
(01)
: 17
-
25
[6]
Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis
Gelman, MS
论文数:
0
引用数:
0
h-index:
0
机构:
Stanford Univ, Dept Biol Sci, Stanford, CA 94305 USA
Gelman, MS
Kopito, RR
论文数:
0
引用数:
0
h-index:
0
机构:
Stanford Univ, Dept Biol Sci, Stanford, CA 94305 USA
Stanford Univ, Dept Biol Sci, Stanford, CA 94305 USA
Kopito, RR
[J].
JOURNAL OF CLINICAL INVESTIGATION,
2002,
110
(11)
: 1591
-
1597
[7]
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
Lewis, HA
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Lewis, HA
Buchanan, SG
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Buchanan, SG
Burley, SK
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Burley, SK
Conners, K
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Conners, K
Dickey, M
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Dickey, M
Dorwart, M
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Dorwart, M
Fowler, R
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Fowler, R
Gao, X
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Gao, X
Guggino, WB
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Guggino, WB
Hendrickson, WA
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Hendrickson, WA
Hunt, JF
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Hunt, JF
Kearins, MC
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Kearins, MC
Lorimer, D
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Lorimer, D
Maloney, PC
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Maloney, PC
Post, KW
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Post, KW
Rajashankar, KR
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Rajashankar, KR
Rutter, ME
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Rutter, ME
Sauder, JM
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Sauder, JM
Shriver, S
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Shriver, S
Thibodeau, PH
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Thibodeau, PH
Thomas, PJ
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Thomas, PJ
Zhang, M
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Zhang, M
Zhao, X
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Zhao, X
Emtage, S
论文数:
0
引用数:
0
h-index:
0
机构:
Struct GenomiX Inc, San Diego, CA 92121 USA
Emtage, S
[J].
EMBO JOURNAL,
2004,
23
(02)
: 282
-
293
[8]
LEWIS HA, 2004, J BIOL CHEM 1103
[9]
The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis
Meacham, GC
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Meacham, GC
Lu, Z
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Lu, Z
King, S
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
King, S
Sorscher, E
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Sorscher, E
Tousson, A
论文数:
0
引用数:
0
h-index:
0
机构:
Univ Alabama, Med Ctr, Dept Cell Biol, Birmingham, AL 35209 USA
Tousson, A
Cyr, DM
论文数:
0
引用数:
0
h-index:
0
机构:
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