Pharmacological chaperones: potential treatment for conformational diseases.

scientific article published in July 2004

Pharmacological chaperones: potential treatment for conformational diseases. is …
instance of (P31):
review articleQ7318358
scholarly articleQ13442814

External links are
P356DOI10.1016/J.TEM.2004.05.003
P8608Fatcat IDrelease_nemvielyfnb7detoavbqwp3jta
P698PubMed publication ID15223052
P5875ResearchGate publication ID8485470

P50authorDaniel-G. BichetQ67522454
Michel BouvierQ38326685
P2093author name stringVirginie Bernier
Monique Lagacé
P433issue5
P921main subjectmolecular chaperonesQ422496
P304page(s)222-228
P577publication date2004-07-01
P1433published inTrends in Endocrinology and MetabolismQ15265727
P1476titlePharmacological chaperones: potential treatment for conformational diseases
P478volume15

Reverse relations

cites work (P2860)
Q3714963714-3-3s are potential biomarkers for HIV-related neurodegeneration
Q91718179A Pharmacochaperone-Based High-Throughput Screening Assay for the Discovery of Chemical Probes of Orphan Receptors
Q37038867A counterintuitive approach to treat enzyme deficiencies: use of enzyme inhibitors for restoring mutant enzyme activity
Q35915766A cure for traffic jams: small molecule chaperones in the endoplasmic reticulum
Q35207863A pharmacogenetic approach to identify mutant forms of α-galactosidase A that respond to a pharmacological chaperone for Fabry disease
Q33629937A phenotypic high throughput screening assay for the identification of pharmacoperones for the gonadotropin releasing hormone receptor
Q55397197A protein folding molecular imaging biosensor monitors the effects of drugs that restore mutant p53 structure and its downstream function in glioblastoma cells.
Q42549992Agonist-induced internalization and downregulation of gonadotropin-releasing hormone receptors
Q41787738Alglucosidase alfa: Long term use in the treatment of patients with Pompe disease
Q34087866Amino acid residues critical for endoplasmic reticulum export and trafficking of platelet-activating factor receptor
Q36317262Analyzing the Effects of a G137V Mutation in the FXN Gene
Q35661403Assay strategies for identification of therapeutic leads that target protein trafficking
Q38533943Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors
Q39944513Cell surface delivery and structural re-organization by pharmacological chaperones of an oligomerization-defective alpha(1b)-adrenoceptor mutant demonstrates membrane targeting of GPCR oligomers
Q37410011Chaperone-like effects of cell-permeant ligands on opioid receptors
Q38957269Chaperones as potential therapeutics for Krabbe disease.
Q33927508Chaperoning G protein-coupled receptors: from cell biology to therapeutics
Q36851855Complementation of a pathogenic IFNGR2 misfolding mutation with modifiers of N-glycosylation.
Q40808463Conformational Stability and Pathogenic Misfolding of the Integral Membrane Protein PMP22.
Q90409111Contribution of Cotranslational Folding Defects to Membrane Protein Homeostasis
Q42321930Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function
Q28297086Developing therapeutic approaches for metachromatic leukodystrophy
Q41486337ER Stress and Iron Homeostasis: A New Frontier for the UPR.
Q35762966Effects of cellular, chemical, and pharmacological chaperones on the rescue of a trafficking-defective mutant of the ATP-binding cassette transporter proteins ABCB1/ABCB4.
Q27002907Emerging novel concept of chaperone therapies for protein misfolding diseases
Q36052285Endoplasmic Reticulum Stress and Unfolded Protein Response Pathways: Potential for Treating Age-related Retinal Degeneration
Q37786731Epileptogenic ion channel mutations: From bedside to bench and, hopefully, back again
Q35075365Escorts take the lead molecular chaperones as therapeutic targets
Q39548423Expression analysis revealing destabilizing mutations in phosphomannomutase 2 deficiency (PMM2-CDG): expression analysis of PMM2-CDG mutations
Q34368512Fabry disease - current treatment and new drug development
Q42426222Fixing cystic fibrosis by correcting CFTR domain assembly
Q34101729Functional and structural analysis of five mutations identified in methylmalonic aciduria cblB type
Q39839857Functional rescue of beta-adrenoceptor dimerization and trafficking by pharmacological chaperones
Q33848408G protein-coupled receptor sorting to endosomes and lysosomes
Q37829756G protein-coupled receptors: walking hand-in-hand, talking hand-in-hand?
Q34140020GABA acts as a ligand chaperone in the early secretory pathway to promote cell surface expression of GABAA receptors
Q64932646Gain of Function for the SCN1A/hNav1.1-L1670W Mutation Responsible for Familial Hemiplegic Migraine.
Q36885398High-throughput screening assays for the identification of chemical probes
Q33275131High-throughput screening for human lysosomal beta-N-Acetyl hexosaminidase inhibitors acting as pharmacological chaperones
Q39890679Identification, characterization and rescue of a novel vasopressin-2 receptor mutation causing nephrogenic diabetes insipidus
Q37071467Increased plasma membrane expression of human follicle-stimulating hormone receptor by a small molecule thienopyr(im)idine
Q39254537Induced Pluripotent Stem Cell Modeling of Gaucher's Disease: What Have We Learned?
Q36734894Inhibition of proteasome activity promotes the correct localization of disease-causing alpha-sarcoglycan mutants in HEK-293 cells constitutively expressing beta-, gamma-, and delta-sarcoglycan
Q42178606Inhibitor mediated protein degradation.
Q38200933Innovative strategies to treat protein misfolding in inborn errors of metabolism: pharmacological chaperones and proteostasis regulators.
Q89621717Involvement of Endoplasmic Reticulum Stress in Palmitate-induced Apoptosis in HepG2 Cells
Q83230136Mechanism of pharmacochaperoning in a mammalian K channel revealed by cryo-EM
Q24299159Mechanisms of pharmacological rescue of trafficking-defective hERG mutant channels in human long QT syndrome
Q81421178Modulatory proteins can rescue a trafficking defective epileptogenic Nav1.1 Na+ channel mutant
Q92989928Molecular Mechanisms and Determinants of Innovative Correction Approaches in Coagulation Factor Deficiencies
Q37238669Molecular and functional characterization of polymorphisms in the secreted phospholipase A2 group X gene: relevance to coronary artery disease
Q80400955Molecular chaperones: the modular evolution of cellular networks
Q26800090Molecular pathophysiology and pharmacology of the voltage-sensing module of neuronal ion channels
Q37351126Mutations in G protein-coupled receptors that impact receptor trafficking and reproductive function.
Q36825595N-glycan structure dictates extension of protein folding or onset of disposal
Q36580003N-linked glycan recognition and processing: the molecular basis of endoplasmic reticulum quality control
Q42705597Neuroprotection by Endoplasmic Reticulum Stress-Induced HRD1 and Chaperones: Possible Therapeutic Targets for Alzheimer's and Parkinson's Disease
Q36646889New therapeutic options for lysosomal storage disorders: enzyme replacement, small molecules and gene therapy
Q37256074Nonfunctional NaV1.1 familial hemiplegic migraine mutant transformed into gain of function by partial rescue of folding defects
Q34685511Norepinephrine deficiency is caused by combined abnormal mRNA processing and defective protein trafficking of dopamine beta-hydroxylase
Q28478533Novel patient cell-based HTS assay for identification of small molecules for a lysosomal storage disease
Q40123566Opioid receptor pharmacological chaperones act by binding and stabilizing newly synthesized receptors in the endoplasmic reticulum
Q37860332Pharmacological chaperone therapy for Gaucher disease: a patent review.
Q35606973Pharmacological chaperones for misfolded gonadotropin-releasing hormone receptors
Q42414938Pharmacological chaperoning: two 'hits' are better than one.
Q21131288Pharmacoperone Identification for Therapeutic Rescue of Misfolded Mutant Proteins
Q50014672Pharmacoperones as Novel Therapeutics for Diverse Protein Conformational Diseases
Q26992276Pharmacoperones: a new therapeutic approach for diseases caused by misfolded G protein-coupled receptors
Q34556955Phenylalanine hydroxylase misfolding and pharmacological chaperones.
Q41955759Phosphorylation-dependent C-terminal binding of 14-3-3 proteins promotes cell surface expression of HIV co-receptor GPR15.
Q39333514Plasma membrane expression of gonadotropin-releasing hormone receptors: regulation by peptide and nonpeptide antagonists
Q47422775Potential Pharmacological Chaperones for Cystathionine Beta-Synthase-Deficient Homocystinuria
Q36510038Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator.
Q58450753Protein Trafficking Diseases
Q39097864Protein aggregation, misfolding and consequential human neurodegenerative diseases.
Q48092471Protein folding: Illuminating chaperone activity
Q36501365Protein misfolding disorders: pathogenesis and intervention
Q46290614Proteostatic Tactics in the Strategy of Sterol Regulation
Q46525116RIC-3 affects properties and quantity of nicotinic acetylcholine receptors via a mechanism that does not require the coiled-coil domains
Q36316416Refolding of misfolded mutant GPCR: post-translational pharmacoperone action in vitro
Q46136957Rescuing proteins of low kinetic stability by chaperones and natural ligands phenylketonuria, a case study
Q37333770Retinitis pigmentosa mutants provide insight into the role of the N-terminal cap in rhodopsin folding, structure, and function
Q36610269Saccharomyces cerevisiae Apn1 mutation affecting stable protein expression mimics catalytic activity impairment: implications for assessing DNA repair capacity in humans
Q51036230Specific inhibition of hamster prion protein translocation by the dodecadepsipeptide valinomycin.
Q38092216Specificity and regulation of the endoplasmic reticulum-associated degradation machinery
Q36791174Stress-induced rearrangements of cellular networks: Consequences for protection and drug design
Q52578645Structural determinants for ligand-receptor conformational selection in a peptide G protein-coupled receptor.
Q35199497Structurally distinct ligands rescue biogenesis defects of the KATP channel complex via a converging mechanism
Q39896634The ART-Rsp5 ubiquitin ligase network comprises a plasma membrane quality control system that protects yeast cells from proteotoxic stress
Q28539680The influence of chemical chaperones on enzymatic activity under thermal and chemical stresses: common features and variation among diverse chemical families
Q39931614The majority of adrenocorticotropin receptor (melanocortin 2 receptor) mutations found in familial glucocorticoid deficiency type 1 lead to defective trafficking of the receptor to the cell surface.
Q28077257The relationship between glucocerebrosidase mutations and Parkinson disease
Q38039520The roles played by highly truncated splice variants of G protein-coupled receptors
Q38211124The therapeutic potential of chemical chaperones in protein folding diseases.
Q21089948Therapeutic rescue of misfolded mutants: validation of primary high throughput screens for identification of pharmacoperone drugs
Q39078944Therapeutic strategies based on modified U1 snRNAs and chaperones for Sanfilippo C splicing mutations
Q36501370Therapy through chaperones: sense or antisense? Cystic fibrosis as a model disease
Q38454078Traffic jam at the sodium channel
Q28755163Trafficking and quality control of the gonadotropin releasing hormone receptor in health and disease
Q37627277Trafficking and signalling of gonadotrophin-releasing hormone receptors: an automated imaging approach
Q26829715Trafficking mechanisms underlying neuronal voltage-gated ion channel localization at the axon initial segment
Q36594595Trafficking of G protein-coupled receptors
Q33699210Trafficking of G-protein-coupled receptors to the plasma membrane: insights for pharmacoperone drugs
Q33717398Transitioning pharmacoperones to therapeutic use: in vivo proof-of-principle and design of high throughput screens.
Q39647606Trimethylamine-N-oxide switches from stabilizing nature: A mechanistic outlook through experimental techniques and molecular dynamics simulation
Q54945677Tuning protein folding in lysosomal storage diseases: the chemistry behind pharmacological chaperones.
Q36697237Unfolding the Therapeutic Potential of Chemical Chaperones for Age-related Macular Degeneration
Q36909424Using EGFP fusions to monitor the functional expression of GPCRs in the Drosophila Schneider 2 cells
Q34494645Using automated imaging to interrogate gonadotrophin-releasing hormone receptor trafficking and function.
Q35093167WNTS and WNT receptors as therapeutic tools and targets in human disease processes.
Q34412182αA-Crystallin-derived mini-chaperone modulates stability and function of cataract causing αAG98R-crystallin

Search more.