Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein.
作者信息
Riordan J R
机构信息
Mayo Foundation and S.C. Johnson Medical Research Center, Mayo Clinic, Scottsdale, AZ 85259, USA.
出版信息
Am J Hum Genet. 1999 Jun;64(6):1499-504. doi: 10.1086/302429.
Abstract
摘要
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From the ER to the golgi: insights from the study of combined factors V and VIII deficiency.从内质网到高尔基体：联合因子V和VIII缺乏症研究的见解
Am J Hum Genet. 1999 Jun;64(6):1493-8. doi: 10.1086/302433.
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A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane K(ATP) channels.一种新的内质网转运信号调节质膜ATP敏感性钾通道的亚基化学计量。
Neuron. 1999 Mar;22(3):537-48. doi: 10.1016/s0896-6273(00)80708-4.
3
The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis.Hdj-2/Hsc70伴侣蛋白对促进CFTR生物合成的早期步骤。
EMBO J. 1999 Mar 15;18(6):1492-505. doi: 10.1093/emboj/18.6.1492.
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Retinal stimulates ATP hydrolysis by purified and reconstituted ABCR, the photoreceptor-specific ATP-binding cassette transporter responsible for Stargardt disease.视网膜可刺激纯化并重组的ABCR水解ATP，ABCR是一种光感受器特异性ATP结合盒转运蛋白，与斯塔加特病有关。
J Biol Chem. 1999 Mar 19;274(12):8269-81. doi: 10.1074/jbc.274.12.8269.
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Evidence that endoplasmic reticulum (ER)-associated degradation of cystic fibrosis transmembrane conductance regulator is linked to retrograde translocation from the ER membrane.内质网（ER）相关的囊性纤维化跨膜传导调节因子降解与从内质网膜逆行转运相关的证据。
J Biol Chem. 1999 Jan 29;274(5):2616-24. doi: 10.1074/jbc.274.5.2616.
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Aggresomes: a cellular response to misfolded proteins.聚集体：细胞对错误折叠蛋白质的一种反应。
J Cell Biol. 1998 Dec 28;143(7):1883-98. doi: 10.1083/jcb.143.7.1883.
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Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome.热休克蛋白90（Hsp90）与新生的囊性纤维化跨膜传导调节因子（CFTR）相互作用受到干扰，会阻止其成熟，并加速蛋白酶体对它的降解。
EMBO J. 1998 Dec 1;17(23):6879-87. doi: 10.1093/emboj/17.23.6879.
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Partial restoration of cAMP-stimulated CFTR chloride channel activity in DeltaF508 cells by deoxyspergualin.脱氧精胍菌素使ΔF508细胞中cAMP刺激的CFTR氯通道活性部分恢复。
Am J Physiol. 1998 Jul;275(1):C171-8. doi: 10.1152/ajpcell.1998.275.1.C171.
9
Delta F508 CFTR localizes in the endoplasmic reticulum-Golgi intermediate compartment in cystic fibrosis cells.ΔF508囊性纤维化跨膜传导调节因子定位于囊性纤维化细胞的内质网-高尔基体中间区室。
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