Using pharmacological chaperones to restore proteostasis (Q33804655)

24747662

29 July 2017

title

Using pharmacological chaperones to restore proteostasis (English)

1 reference

29 July 2017

0 references

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24747662%20AND%20SRC:MED&resulttype=core&format=json

31 May 2020

Ya-Juan Wang

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31 May 2020

Xiao-Jing Di

series ordinal

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31 May 2020

publication date

18 April 2014

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24747662

29 July 2017

published in

Pharmacological Research

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31 May 2020

volume

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24747662%20AND%20SRC:MED&resulttype=core&format=json

31 May 2020

page(s)

3-9

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24747662%20AND%20SRC:MED&resulttype=core&format=json

Glycoprotein folding and quality-control mechanisms in protein-folding diseases

31 May 2020

cites work

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Chemical kinetics for drug discovery to combat protein aggregation diseases

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Lysosomal storage disorders: old diseases, present and future challenges

4 July 2018

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Nicotine exploits a COPI-mediated process for chaperone-mediated up-regulation of its receptors.

4 July 2018

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A thermodynamic assay to test pharmacological chaperones for Fabry disease.

4 July 2018

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Restoration of testis function in hypogonadotropic hypogonadal mice harboring a misfolded GnRHR mutant by pharmacoperone drug therapy

4 July 2018

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SAHA enhances Proteostasis of epilepsy-associated α1(A322D)β2γ2 GABA(A) receptors.

4 July 2018

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Proteostasis and aging of stem cells

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Identification of GABA(C) receptor protein homeostasis network components from three tandem mass spectrometry proteomics approaches.

4 July 2018

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Gating of pentameric ligand-gated ion channels: structural insights and ambiguities

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Neuronopathic lysosomal storage diseases: clinical and pathologic findings.

4 July 2018

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Molecular chaperone functions in protein folding and proteostasis

4 July 2018

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Protein folding in the endoplasmic reticulum

4 July 2018

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N-linked protein glycosylation in the ER.

4 July 2018

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Glucocerebrosidase inhibitors for the treatment of Gaucher disease.

4 July 2018

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Gaucher disease: a comprehensive review

4 July 2018

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Diversity in the origins of proteostasis networks--a driver for protein function in evolution

4 July 2018

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Protein analysis by shotgun/bottom-up proteomics

4 July 2018

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FKBP10 depletion enhances glucocerebrosidase proteostasis in Gaucher disease fibroblasts.

4 July 2018

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Novel pharmacological strategies to treat cystic fibrosis.

4 July 2018

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Pharmacological chaperones as therapeutics for lysosomal storage diseases

4 July 2018

1 reference

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Gaucher disease and other storage disorders

4 July 2018

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Cleaning up: ER-associated degradation to the rescue

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Pharmacological chaperones correct misfolded GPCRs and rescue function: protein trafficking as a therapeutic target

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Flagging and docking: dual roles for N-glycans in protein quality control and cellular proteostasis

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Gaucher disease paradigm: from ERAD to comorbidity

4 July 2018

1 reference

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Ion channel mutations in neuronal diseases: a genetics perspective

4 July 2018

1 reference

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The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Pharmacological chaperoning of nicotinic acetylcholine receptors reduces the endoplasmic reticulum stress response.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Defective trafficking of rhodopsin and its role in retinal degenerations

4 July 2018

1 reference

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The transthyretin amyloidoses: from delineating the molecular mechanism of aggregation linked to pathology to a regulatory-agency-approved drug

4 July 2018

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Pharmacological chaperone therapy for Fabry disease

4 July 2018

1 reference

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The Molecular Basis of Pharmacological Chaperoning in Human α-Galactosidase

4 July 2018

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COPII and the regulation of protein sorting in mammals

4 July 2018

1 reference

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CFTR: folding, misfolding and correcting the ΔF508 conformational defect.

4 July 2018

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The unfolded protein response: from stress pathway to homeostatic regulation

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

COPII and COPI traffic at the ER-Golgi interface

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Pharmacological Chaperones for Misfolded Gonadotropin-Releasing Hormone Receptors

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Chemical and Biological Approaches for Adapting Proteostasis to Ameliorate Protein Misfolding and Aggregation Diseases-Progress and Prognosis

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Molecular chaperones in protein folding and proteostasis

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Oligosaccharyltransferase: the central enzyme of N‐linked protein glycosylation

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Identification and Characterization of Pharmacological Chaperones to Correct Enzyme Deficiencies in Lysosomal Storage Disorders

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

The stress of protein misfolding: from single cells to multicellular organisms

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Emergent Properties of Proteostasis in Managing Cystic Fibrosis

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Identification of a NBD1-binding pharmacological chaperone that corrects the trafficking defect of F508del-CFTR.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Nicotine up-regulates alpha4beta2 nicotinic receptors and ER exit sites via stoichiometry-dependent chaperoning

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Emergent properties of proteostasis-COPII coupled systems in human health and disease

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

GABA acts as a ligand chaperone in the early secretory pathway to promote cell surface expression of GABAA receptors

4 July 2018

1 reference

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Endoplasmic reticulum Ca2+ increases enhance mutant glucocerebrosidase proteostasis.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Potential of nonpeptide (ant)agonists to rescue vasopressin V2 receptor mutants for the treatment of X-linked nephrogenic diabetes insipidus

4 July 2018

1 reference

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Molecular mechanisms of rhodopsin retinitis pigmentosa and the efficacy of pharmacological rescue

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Enhancement of the surface expression of G protein-coupled receptors.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Glycoprotein folding, quality control and ER-associated degradation

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Biological and chemical approaches to diseases of proteostasis deficiency

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Nicotine is a selective pharmacological chaperone of acetylcholine receptor number and stoichiometry. Implications for drug discovery.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Chemical and biological approaches synergize to ameliorate protein-folding diseases.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Adapting proteostasis for disease intervention

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasis

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Pharmacologic chaperoning as a strategy to treat Gaucher disease

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

G protein-coupled receptor trafficking in health and disease: lessons learned to prepare for therapeutic mutant rescue in vivo

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Functional rescue of vasopressin V2 receptor mutants in MDCK cells by pharmacochaperones: relevance to therapy of nephrogenic diabetes insipidus

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Rescue of folding defects in ABC transporters using pharmacological chaperones

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Molecular chaperones and protein quality control

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Versatility of the endoplasmic reticulum protein folding factory

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

The mammalian unfolded protein response

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Pharmacological chaperone action on G-protein-coupled receptors.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Biology of cardiac arrhythmias: ion channel protein trafficking

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Cys-loop receptors: new twists and turns

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

A contradictory treatment for lysosomal storage disorders: inhibitors enhance mutant enzyme activity

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

The binding site for channel blockers that rescue misprocessed human long QT syndrome type 2 ether-a-gogo-related gene (HERG) mutations

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Autophagy as a regulated pathway of cellular degradation

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

The Hsp70 and Hsp60 Chaperone Machines

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Molecular chaperones in cellular protein folding

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

4 July 2018

The heat-shock response

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

A Phase 2 study of migalastat hydrochloride in females with Fabry disease: selection of population, safety and pharmacodynamic effects.

4 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Click chemistry approach to new N-substituted aminocyclitols as potential pharmacological chaperones for Gaucher disease

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

2,5-Dideoxy-2,5-imino-d-altritol as a new class of pharmacological chaperone for Fabry disease.

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Chemical chaperones and permissive temperatures alter localization of Gaucher disease associated glucocerebrosidase variants

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Pharmacologic chaperones as a potential treatment for X-linked nephrogenic diabetes insipidus

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Retinoids assist the cellular folding of the autosomal dominant retinitis pigmentosa opsin mutant P23H.

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

Different binding orientations for the same agonist at homologous receptors: a lock and key or a simple wedge?

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

30 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4070435

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

30 October 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/24747662

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1016/J.PHRS.2014.04.002

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24747662%20AND%20SRC:MED&resulttype=core&format=json

release_t3to3qvqn5flvob4dkhyqkwsle

31 May 2020

Fatcat ID

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/t3to3qvqn5flvob4dkhyqkwsle

mapped directly with Wikidata item

24 November 2022

based on heuristic

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24747662%20AND%20SRC:MED&resulttype=core&format=json

31 May 2020

24747662

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24747662%20AND%20SRC:MED&resulttype=core&format=json