Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis.
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Correcting the cystic fibrosis disease mutant, A455E CFTRMolecular Chaperones as Targets to Circumvent the CFTR Defect in Cystic FibrosisCystic Fibrosis: Lessons from the Sweat GlandA Golgi-associated PDZ domain protein modulates cystic fibrosis transmembrane regulator plasma membrane expressionTrafficking of TRPP2 by PACS proteins represents a novel mechanism of ion channel regulationMyosin VI regulates endocytosis of the cystic fibrosis transmembrane conductance regulatorIdentification and subcellular localization of human rab5b, a new member of the ras-related superfamily of GTPasesRescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustatExpression of the MRP gene-encoded conjugate export pump in liver and its selective absence from the canalicular membrane in transport-deficient mutant hepatocytesDeletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperoneEndoplasmic reticulum protein quality control is determined by cooperative interactions between Hsp/c70 protein and the CHIP E3 ligaseSLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epitheliaThe human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70Rhmod syndrome: a family study of the translation-initiator mutation in the Rh50 glycoprotein gene.Differential effects of Hsc70 and Hsp70 on the intracellular trafficking and functional expression of epithelial sodium channelsDigitoxin mimics gene therapy with CFTR and suppresses hypersecretion of IL-8 from cystic fibrosis lung epithelial cellsAn aquaporin-2 water channel mutant which causes autosomal dominant nephrogenic diabetes insipidus is retained in the Golgi complexInhibition of cystic fibrosis transmembrane conductance regulator by novel interaction with the metabolic sensor AMP-activated protein kinaseMolecular pathogenesis of Wilson and Menkes disease: correlation of mutations with molecular defects and disease phenotypesGlycosylation and the cystic fibrosis transmembrane conductance regulatorCFTR gating II: Effects of nucleotide binding on the stability of open statesTethered and Polymer Supported Bilayer Lipid Membranes: Structure and FunctionRegulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity.Airway epithelial repair in health and disease: Orchestrator or simply a player?Targeted therapies to improve CFTR function in cystic fibrosisDefining the blanks--pharmacochaperoning of SLC6 transporters and ABC transportersMechanisms for quality control of misfolded transmembrane proteinsThe Evolution of Cystic Fibrosis CareWrecked regulation of intrinsically disordered proteins in diseases: pathogenicity of deregulated regulatorsThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyStructure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulatorRequirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved SequencesThe a-factor transporter (STE6 gene product) and cell polarity in the yeast Saccharomyces cerevisiae.Der3p/Hrd1p is required for endoplasmic reticulum-associated degradation of misfolded lumenal and integral membrane proteins.Distinct roles for the Hsp40 and Hsp90 molecular chaperones during cystic fibrosis transmembrane conductance regulator degradation in yeast.Mutations within the first LSGGQ motif of Ste6p cause defects in a-factor transport and mating in Saccharomyces cerevisiae.The yeast ubr1 ubiquitin ligase participates in a prominent pathway that targets cytosolic thermosensitive mutants for degradationCystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectivesCFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisPharmacological chaperones rescue cell-surface expression and function of misfolded V2 vasopressin receptor mutants
P2860
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P2860
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis.
description
1990 nî lūn-bûn
@nan
1990年の論文
@ja
1990年論文
@yue
1990年論文
@zh-hant
1990年論文
@zh-hk
1990年論文
@zh-mo
1990年論文
@zh-tw
1990年论文
@wuu
1990年论文
@zh
1990年论文
@zh-cn
name
Defective intracellular transp ...... basis of most cystic fibrosis.
@en
type
label
Defective intracellular transp ...... basis of most cystic fibrosis.
@en
prefLabel
Defective intracellular transp ...... basis of most cystic fibrosis.
@en
P2093
P1433
P1476
Defective intracellular transp ...... basis of most cystic fibrosis.
@en
P2093
Gregory RJ
Marshall J
O'Riordan CR
P304
P356
10.1016/0092-8674(90)90148-8
P407
P577
1990-11-01T00:00:00Z