(Q34305593)

1 August 2017

title

A genomic signature approach to rescue ΔF508-cystic fibrosis transmembrane conductance regulator biosynthesis and function (English)

1 reference

1 August 2017

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Paul B McCray

5

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author name string

Shyam Ramachandran

1

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1 August 2017

Samantha R Osterhaus

2

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1 August 2017

Philip H Karp

3

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1 August 2017

Michael J Welsh

4

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1 August 2017

publication date

1 September 2014

1 reference

American Journal of Respiratory Cell and Molecular Biology

1 August 2017

published in

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1 August 2017

volume

51

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1 August 2017

issue

3

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1 August 2017

page(s)

354-362

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Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors

1 August 2017

cites work

1 reference

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Mechanism-based corrector combination restores ΔF508-CFTR folding and function

6 July 2018

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Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein

6 July 2018

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Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells

6 July 2018

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6 July 2018

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A gene expression signature of emphysema-related lung destruction and its reversal by the tripeptide GHK

6 July 2018

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Comparative gene expression profiling of benign and malignant lesions reveals candidate therapeutic compounds for leiomyosarcoma

6 July 2018

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A microRNA network regulates expression and biosynthesis of wild-type and DeltaF508 mutant cystic fibrosis transmembrane conductance regulator

6 July 2018

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Gene expression-based chemical genomics identifies heat-shock protein 90 inhibitors as potential therapeutic drugs in cholangiocarcinoma.

6 July 2018

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Expression-based genome-wide association study links the receptor CD44 in adipose tissue with type 2 diabetes

6 July 2018

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Pharmacological rescue of the mutant cystic fibrosis transmembrane conductance regulator (CFTR) detected by use of a novel fluorescence platform

6 July 2018

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

6 July 2018

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6 July 2018

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6 July 2018

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Nucleosome occupancy reveals regulatory elements of the CFTR promoter.

6 July 2018

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Computational repositioning of the anticonvulsant topiramate for inflammatory bowel disease

6 July 2018

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Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.

6 July 2018

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Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling.

6 July 2018

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Cyanoquinolines with independent corrector and potentiator activities restore ΔPhe508-cystic fibrosis transmembrane conductance regulator chloride channel function in cystic fibrosis

6 July 2018

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mRNA expression signatures of human skeletal muscle atrophy identify a natural compound that increases muscle mass

6 July 2018

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6 July 2018

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Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation

6 July 2018

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Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening

6 July 2018

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The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis.

6 July 2018

1 reference

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Peripheral protein quality control removes unfolded CFTR from the plasma membrane

6 July 2018

1 reference

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Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Predicting new molecular targets for known drugs

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

GeneChaser: identifying all biological and clinical conditions in which genes of interest are differentially expressed

6 July 2018

1 reference

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RETRACTED: A genomic approach to colon cancer risk stratification yields biologic insights into therapeutic opportunities

6 July 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Insulin-like growth factor-I activates gene transcription programs strongly associated with poor breast cancer prognosis

6 July 2018

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Estrogen-dependent signaling in a molecularly distinct subclass of aggressive prostate cancer

6 July 2018

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Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Gene expression signature-based chemical genomic prediction identifies a novel class of HSP90 pathway modulators

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Gene expression-based chemical genomics identifies rapamycin as a modulator of MCL1 and glucocorticoid resistance

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

The Connectivity Map: using gene-expression signatures to connect small molecules, genes, and disease

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Gene expression profile analysis of 4-phenylbutyrate treatment of IB3-1 bronchial epithelial cell line demonstrates a major influence on heat-shock proteins

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

An immortalized cystic fibrosis tracheal epithelial cell line homozygous for the delta F508 CFTR mutation

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4189493

Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids

6 July 2018

1 reference

Crossref

https://api.crossref.org/works/10.1165%2FRCMB.2014-0007OC

Meta-basic estimates the size of druggable human genome

21 January 2018

1 reference

Crossref

https://api.crossref.org/works/10.1165%2FRCMB.2014-0007OC

21 January 2018

Identifiers

DOI

10.1165/RCMB.2014-0007OC

1 reference

1 August 2017

1 reference

1 August 2017

1 reference