4-Phenylbutyrate attenuates the ER stress response and cyclic AMP accumulation in DYT1 dystonia cell models (Q34472800)

2 August 2017

4-Phenylbutyrate attenuates the ER stress response and cyclic AMP accumulation in DYT1 dystonia cell models (English)

1 reference

2 August 2017

1 reference

Wayne I Lencer

4

0 references

author name string

Jin A Cho

1

1 reference

2 August 2017

Xuan Zhang

2

1 reference

2 August 2017

Gregory M Miller

3

1 reference

2 August 2017

Flavia C Nery

5

1 reference

2 August 2017

language of work or name

English

0 references

publication date

7 November 2014

1 reference

2 August 2017

1 reference

2 August 2017

9

1 reference

2 August 2017

11

1 reference

2 August 2017

e110086

1 reference

Creative Commons Attribution 4.0 International

2 August 2017

start time

7 November 2014

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

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TorsinA participates in endoplasmic reticulum-associated degradation

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The early-onset torsion dystonia-associated protein, torsinA, is a homeostatic regulator of endoplasmic reticulum stress response

6 July 2018

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Chemical enhancement of torsinA function in cell and animal models of torsion dystonia

6 July 2018

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Multiple roles of HDAC inhibition in neurodegenerative conditions.

6 July 2018

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TorsinA binds the KASH domain of nesprins and participates in linkage between nuclear envelope and cytoskeleton

6 July 2018

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ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.

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Activation of tyrosine hydroxylase mRNA translation by cAMP in midbrain dopaminergic neurons

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siRNA knock-down of mutant torsinA restores processing through secretory pathway in DYT1 dystonia cells

6 July 2018

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The dystonia-associated protein torsinA modulates synaptic vesicle recycling

6 July 2018

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The cyclic AMP cascade is altered in the fragile X nervous system

6 July 2018

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Proteasome function is regulated by cyclic AMP-dependent protein kinase through phosphorylation of Rpt6

6 July 2018

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Mutant torsinA interferes with protein processing through the secretory pathway in DYT1 dystonia cells

6 July 2018

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SGCE missense mutations that cause myoclonus-dystonia syndrome impair epsilon-sarcoglycan trafficking to the plasma membrane: modulation by ubiquitination and torsinA

6 July 2018

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Motor deficits and hyperactivity in Dyt1 knockdown mice.

6 July 2018

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

6 July 2018

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Endoplasmic reticulum stress in health and disease

6 July 2018

1 reference

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

6 July 2018

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The unfolded protein response: a stress signaling pathway critical for health and disease.

6 July 2018

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6 July 2018

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Loss of the dystonia-associated protein torsinA selectively disrupts the neuronal nuclear envelope

6 July 2018

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Dystonia-causing mutant torsinA inhibits cell adhesion and neurite extension through interference with cytoskeletal dynamics

6 July 2018

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Therapy of dystonia in Japan

6 July 2018

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Generation and characterization of Dyt1 DeltaGAG knock-in mouse as a model for early-onset dystonia

6 July 2018

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Role of p97 AAA-ATPase in the retrotranslocation of the cholera toxin A1 chain, a non-ubiquitinated substrate

6 July 2018

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Impaired motor learning in mice expressing torsinA with the DYT1 dystonia mutation.

6 July 2018

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Effect of torsinA on membrane proteins reveals a loss of function and a dominant-negative phenotype of the dystonia-associated DeltaE-torsinA mutant

6 July 2018

1 reference

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Down-regulation of nitrergic transmission in the rat striatum after chronic nigrostriatal deafferentation

6 July 2018

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Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

6 July 2018

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Phenylbutyrate increases SMN expression in vitro: relevance for treatment of spinal muscular atrophy

6 July 2018

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Developments in the molecular biology of DYT1 dystonia

6 July 2018

1 reference

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Dysfunction of dopaminergic pathways in dystonia

6 July 2018

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Surgical therapy for dystonia

6 July 2018

1 reference

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Chemical chaperones: a pharmacological strategy for disorders of protein folding and trafficking

6 July 2018

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Medical treatment of dystonia

6 July 2018

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Life extension in Drosophila by feeding a drug.

6 July 2018

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Characterization of GTP cyclohydrolase I gene expression in the human neuroblastoma SKN-BE(2)M17: enhanced transcription in response to cAMP is conferred by the proximal promoter

6 July 2018

1 reference

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Cervical dystonia is associated with a polymorphism in the dopamine (D5) receptor gene

6 July 2018

1 reference

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A close association of torsinA and alpha-synuclein in Lewy bodies: a fluorescence resonance energy transfer study

6 July 2018

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Diazepam and rolipram differentially inhibit cyclic AMP-specific phosphodiesterases PDE4A1 and PDE4B3 in the mouse

6 July 2018

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Dopa-responsive dystonia simulating spastic paraplegia due to tyrosine hydroxylase (TH) gene mutations

6 July 2018

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Alternative pathway therapy for urea cycle disorders: twenty years later

6 July 2018

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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

6 July 2018

1 reference

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6 July 2018

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Dopamine receptors: from structure to function

6 July 2018

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6 July 2018

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The early-onset torsion dystonia gene (DYT1) encodes an ATP-binding protein

27 October 2018

1 reference

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Long-term treatment of girls with ornithine transcarbamylase deficiency

6 July 2018

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Hereditary progressive dystonia with marked diurnal fluctuation caused by mutations in the GTP cyclohydrolase I gene

6 July 2018

1 reference

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Torsion dystonia: a double-blind, prospective trial of high-dosage trihexyphenidyl

6 July 2018

1 reference

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Dopamine transmission in DYT1 dystonia: a biochemical and autoradiographical study

6 July 2018

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Activation of silent and weak synapses by cAMP-dependent protein kinase in cultured cerebellar granule neurons.

21 January 2018

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Learning and memory deficits consequent to reduction of the fragile X mental retardation protein result from metabotropic glutamate receptor-mediated inhibition of cAMP signaling in Drosophila.

21 January 2018

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Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice

21 January 2018

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Cyclic nucleotide research -- still expanding after half a century

21 January 2018

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Identification of a specific assembly of the g protein golf as a critical and regulated module of dopamine and adenosine-activated cAMP pathways in the striatum

21 January 2018

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Targeted transgene integration into transgenic mouse fibroblasts carrying the full-length human AAVS1 locus mediated by HSV/AAV rep(+) hybrid amplicon vector.

21 January 2018

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PET imaging of the pre-synaptic dopamine uptake sites in rapid-onset dystonia-parkinsonism (RDP).

21 January 2018

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Imaging the pre- and postsynaptic side of striatal dopaminergic synapses in idiopathic cervical dystonia: a SPECT study using [123I] epidepride and [123I] beta-CIT.

21 January 2018

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Effects of the chemical chaperone 4-phenylbutylate on the function of the serotonin transporter (SERT) expressed in COS-7 cells.

21 January 2018

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Sodium 4-phenylbutyrate protects against cerebral ischemic injury.

21 January 2018

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Phenylbutyrate rescues dendritic spine loss associated with memory deficits in a mouse model of Alzheimer disease

21 January 2018

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Neurodegeneration of mouse nigrostriatal dopaminergic system induced by repeated oral administration of rotenone is prevented by 4-phenylbutyrate, a chemical chaperone.

21 January 2018

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Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease

21 January 2018

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Combined riluzole and sodium phenylbutyrate therapy in transgenic amyotrophic lateral sclerosis mice

21 January 2018

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Dopamine transmission in DYT1 dystonia

21 January 2018

1 reference

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Stimulation of forskolin of intact S49 lymphoma cells involves the nucleotide regulatory protein of adenylate cyclase

27 October 2018

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Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR

27 October 2018

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12 December 2020

Effect of Cyclic AMP-Dependent Protein Phosphorylating Conditions on the pH-Dependent Activity of Tyrosine Hydroxylase from Beef and Rat Striata

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12 December 2020

Mutations among Italian mucopolysaccharidosis type I patients

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12 December 2020

Decreased [18F]spiperone binding in putamen in idiopathic focal dystonia

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12 December 2020

Cathechol‐O‐methyltransferase activity in cultured human skin fibroblasts from controls and patients with dystonia musculorum deformans

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12 December 2020

10.1371/JOURNAL.PONE.0110086

Identifiers

DOI

1 reference

2 August 2017

0 references

1 reference

2 August 2017

1 reference