(Q36863386)

16 August 2017

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title

Chemical and pharmacological chaperones as new therapeutic agents (English)

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16 August 2017

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Tip W Loo

1

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16 August 2017

David M Clarke

series ordinal

2

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16 August 2017

publication date

28 June 2007

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Expert Reviews in Molecular Medicine

16 August 2017

published in

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16 August 2017

volume

9

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16 August 2017

issue

16

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16 August 2017

page(s)

1-18

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The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants.

16 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Cystic fibrosis transmembrane conductance regulator has an altered structure when its maturation is inhibited

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Insertion of an arginine residue into the transmembrane segments corrects protein misfolding

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

The transmembrane domains of the human multidrug resistance P-glycoprotein are sufficient to mediate drug binding and trafficking to the cell surface.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Superfolding of the partially unfolded core-glycosylated intermediate of human P-glycoprotein into the mature enzyme is promoted by substrate-induced transmembrane domain interactions.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Recent progress in understanding the mechanism of P-glycoprotein-mediated drug efflux.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Evolution of the ATP-binding cassette (ABC) transporter superfamily in vertebrates

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Chemical chaperones reduce ER stress and restore glucose homeostasis in a mouse model of type 2 diabetes

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Suppressive effects of 4-phenylbutyrate on the aggregation of Pael receptors and endoplasmic reticulum stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Nonylphenol ethoxylates, but not nonylphenol, are substrates of the human multidrug resistance P-glycoprotein

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Mistargeted MRPdeltaF728 mutant is rescued by intracellular GSH.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Functional characterization of Candida albicans ABC transporter Cdr1p.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Mutational analysis of ABCG2: role of the GXXXG motif

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Appropriate polarization following pharmacological rescue of V2 vasopressin receptors encoded by X-linked nephrogenic diabetes insipidus alleles involves a conformation of the receptor that also attains mature glycosylation

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Improvement in cardiac function in the cardiac variant of Fabry's disease with galactose-infusion therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Defective aquaporin-2 trafficking in nephrogenic diabetes insipidus and correction by chemical chaperones

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Pharmacological enhancement of beta-hexosaminidase activity in fibroblasts from adult Tay-Sachs and Sandhoff Patients.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Chemical chaperone therapy for brain pathology in G(M1)-gangliosidosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Gaucher disease-associated glucocerebrosidases show mutation-dependent chemical chaperoning profiles

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Chemical chaperones increase the cellular activity of N370S beta -glucosidase: a therapeutic strategy for Gaucher disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

A synthetic chaperone corrects the trafficking defect and disease phenotype in a protein misfolding disorder

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Trehalose-enzyme interactions result in structure stabilization and activity inhibition. The role of viscosity

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Mechanism of protein stabilization by glycerol: preferential hydration in glycerol-water mixtures

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Small molecule pharmacological chaperones: From thermodynamic stabilization to pharmaceutical drugs.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

The DeltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Characterization of two alpha-galactosidase mutants (Q279E and R301Q) found in an atypical variant of Fabry disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Introduction of the most common cystic fibrosis mutation (Delta F508) into human P-glycoprotein disrupts packing of the transmembrane segments.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Galactose stabilizes various missense mutants of alpha-galactosidase in Fabry disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Biosynthesis and processing of epithelial sodium channels in Xenopus oocytes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Characterization of a mutant alpha-galactosidase gene product for the late-onset cardiac form of Fabry disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Impaired trafficking of distal renal tubular acidosis mutants of the human kidney anion exchanger kAE1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Therapeutic strategies to ameliorate lysosomal storage disorders--a focus on Gaucher disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

The cell biology of lysosomal storage disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Pharmacochaperones post-translationally enhance cell surface expression by increasing conformational stability of wild-type and mutant vasopressin V2 receptors

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Nephrogenic diabetes insipidus.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Nephrogenic Diabetes Insipidus

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Proper folding and endoplasmic reticulum to golgi transport of tyrosinase are induced by its substrates, DOPA and tyrosine

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Inhibition of Hedgehog signaling by direct binding of cyclopamine to Smoothened

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Correction of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effects

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Analysis of early events in acetylcholine receptor assembly

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Export from the endoplasmic reticulum represents the limiting step in the maturation and cell surface expression of the human delta opioid receptor

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Cystic fibrosis: channel, catalytic, and folding properties of the CFTR protein.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

A mutation linked with autism reveals a common mechanism of endoplasmic reticulum retention for the alpha,beta-hydrolase fold protein family

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Integration of endoplasmic reticulum signaling in health and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Ligands act as pharmacological chaperones and increase the efficiency of delta opioid receptor maturation

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Traffic jams II: an update of diseases of intracellular transport

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Pharmacological chaperone-mediated in vivo folding and stabilization of the P23H-opsin mutant associated with autosomal dominant retinitis pigmentosa

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Amyloids, prions and the inherent infectious nature of misfolded protein aggregates

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Protein misfolding and aggregation: new examples in medicine and biology of the dark side of the protein world

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

G-protein-coupled receptor trafficking: understanding the chemical basis of health and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Pharmacological chaperone action on G-protein-coupled receptors.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Sulfonylureas correct trafficking defects of disease-causing ATP-sensitive potassium channels by binding to the channel complex

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Sulfonylureas correct trafficking defects of ATP-sensitive potassium channels caused by mutations in the sulfonylurea receptor

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Defective trafficking and function of KATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

The C terminus of SUR1 is required for trafficking of KATP channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

A conditional mutation affecting localization of the Menkes disease copper ATPase. Suppression by copper supplementation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Pharmacological rescue of carnitine transport in primary carnitine deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Recovering antibody secretion using a hapten ligand as a chemical chaperone.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

A nonpeptide antagonist reveals a highly glycosylated state of the rabbit kinin B1 receptor.

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Ligand binding is a critical requirement for plasma membrane expression of heteromeric kainate receptors

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Folding efficiency is rate-limiting in dopamine D4 receptor biogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Identification and pharmacological correction of a membrane trafficking defect associated with a mutation in the sulfonylurea receptor causing familial hyperinsulinism

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Calcium-pump inhibitors induce functional surface expression of Delta F508-CFTR protein in cystic fibrosis epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Cotranslocational degradation: utilitarianism in the ER stress response

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

The mammalian unfolded protein response

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

The biological and chemical basis for tissue-selective amyloid disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

GDAP1, the protein causing Charcot-Marie-Tooth disease type 4A, is expressed in neurons and is associated with mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

ER quality control: the cytoplasmic connection

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

The glycan code of the endoplasmic reticulum: asparagine-linked carbohydrates as protein maturation and quality-control tags

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Predicting the effects of amino acid substitutions on protein function

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Human Gene Mutation Database (HGMD): 2003 update

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Correction of Defective Protein Kinesis of Human P-glycoprotein Mutants by Substrates and Modulators

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Modulating the Folding of P-Glycoprotein and Cystic Fibrosis Transmembrane Conductance Regulator Truncation Mutants with Pharmacological Chaperones

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

Pharmacological rescue of mutant p53 conformation and function

21 January 2018

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Protein misfolding and human disease

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Novel therapeutic strategies for the treatment of protein-misfolding diseases

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Pharmacological chaperones rescue cell-surface expression and function of misfolded V2 vasopressin receptor mutants

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

In vitro inhibition and intracellular enhancement of lysosomal α-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Nicotine acts as a pharmacological chaperone to up-regulate human alpha4beta2 acetylcholine receptors

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Protein aggregation in disease: a role for folding intermediates forming specific multimeric interactions

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Chemical Chaperones: Mechanisms of Action and Potential Use

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Rescue of hypogonadotropic hypogonadism-causing and manufactured GnRH receptor mutants by a specific protein-folding template: misrouted proteins as a novel disease etiology and therapeutic target

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Association and stoichiometry of K(ATP) channel subunits

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

Partial restoration of defective chloride conductance in DeltaF508 CF mice by trimethylamine oxide

1 reference

https://api.crossref.org/works/10.1017%2FS1462399407000361

7 January 2021

10.1017/S1462399407000361

Identifiers

DOI

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1 reference