Pharmacological modulation of the retinal unfolded protein response in Bardet-Biedl syndrome reduces apoptosis and preserves light detection ability (Q28591411)

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Language	Label	Description	Also known as
English	Pharmacological modulation of the retinal unfolded protein response in Bardet-Biedl syndrome reduces apoptosis and preserves light detection ability	scientific journal article

Statements

scholarly article

1 reference

31 January 2017

Pharmacological modulation of the retinal unfolded protein response in Bardet-Biedl syndrome reduces apoptosis and preserves light detection ability (English)

1 reference

31 January 2017

apoptotic process

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intraciliary transport

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Gene Ontology release 2020-05-02

Gene Ontology ID

S-antigen, retina and pineal gland (arrestin)

1 reference

GOA release 2020-03-11

Bardet-Biedl syndrome 12 (human)

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GOA release 2020-03-11

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GOA release 2020-03-11

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based on heuristic

inferred from title

Bardet-Biedl syndrome

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based on heuristic

inferred from title

Hélène Dollfus

7

object named as

Hélène Dollfus

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31 January 2017

author name string

Anais Mockel

1

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31 January 2017

Cathy Obringer

2

1 reference

31 January 2017

Theodorus B. M. Hakvoort

3

1 reference

31 January 2017

Mathias Seeliger

4

1 reference

31 January 2017

Wouter H. Lamers

5

1 reference

31 January 2017

Corinne Stoetzel

6

1 reference

31 January 2017

Vincent Marion

8

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31 January 2017

language of work or name

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publication date

26 October 2012

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31 January 2017

Journal of Biological Chemistry

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31 January 2017

287

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31 January 2017

44

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31 January 2017

37483–37494

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31 January 2017

BBS-induced ciliary defect enhances adipogenesis, causing paradoxical higher-insulin sensitivity, glucose usage, and decreased inflammatory response

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Intrinsic protein-protein interaction-mediated and chaperonin-assisted sequential assembly of stable bardet-biedl syndrome protein complex, the BBSome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Molecular and therapeutic potential and toxicity of valproic acid

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Identification and functional analysis of the vision-specific BBS3 (ARL6) long isoform

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

AHI1 is required for photoreceptor outer segment development and is a modifier for retinal degeneration in nephronophthisis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

BBS6, BBS10, and BBS12 form a complex with CCT/TRiC family chaperonins and mediate BBSome assembly

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Divergent effects of PERK and IRE1 signaling on cell viability

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

ERdj5 is required as a disulfide reductase for degradation of misfolded proteins in the ER

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Antihypertensive drug guanabenz is active in vivo against both yeast and mammalian prions

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

A knockin mouse model of the Bardet-Biedl syndrome 1 M390R mutation has cilia defects, ventriculomegaly, retinopathy, and obesity

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Impaired photoreceptor protein transport and synaptic transmission in a mouse model of Bardet-Biedl syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

A core complex of BBS proteins cooperates with the GTPase Rab8 to promote ciliary membrane biogenesis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Signal integration in the endoplasmic reticulum unfolded protein response

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

The Ciliopathies: An Emerging Class of Human Genetic Disorders

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Mkks-null mice have a phenotype resembling Bardet-Biedl syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Bbs2-null mice have neurosensory deficits, a defect in social dominance, and retinopathy associated with mislocalization of rhodopsin

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Bardet-Biedl syndrome type 4 (BBS4)-null mice implicate Bbs4 in flagella formation but not global cilia assembly

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

XBP1 mRNA is induced by ATF6 and spliced by IRE1 in response to ER stress to produce a highly active transcription factor

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Plasma cell differentiation requires the transcription factor XBP-1

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17 March 2017

Regulated translation initiation controls stress-induced gene expression in mammalian cells

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17 March 2017

Presenilin-1 mutations downregulate the signalling pathway of the unfolded-protein response

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

The interferon-induced double-stranded RNA-activated protein kinase PKR will phosphorylate serine, threonine, or tyrosine at residue 51 in eukaryotic initiation factor 2alpha

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Protein translation and folding are coupled by an endoplasmic-reticulum-resident kinase

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

Cytochrome c and dATP-dependent formation of Apaf-1/caspase-9 complex initiates an apoptotic protease cascade

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

17 March 2017

When cilia go bad: cilia defects and ciliopathies

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

7 April 2017

Integrating the mechanisms of apoptosis induced by endoplasmic reticulum stress

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

7 April 2017

IRE1 signaling affects cell fate during the unfolded protein response

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

7 April 2017

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

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

7 April 2017

ER stress in retinal degeneration in S334ter Rho rats

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

29 September 2017

Β-adrenergic receptor stimulation induces endoplasmic reticulum stress in adult cardiac myocytes: role in apoptosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

29 September 2017

IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsin

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

29 September 2017

TUDCA slows retinal degeneration in two different mouse models of retinitis pigmentosa and prevents obesity in Bardet-Biedl syndrome type 1 mice

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

29 September 2017

ER stress in retinal degeneration: a target for rational therapy?

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

29 September 2017

Retinal dystrophy in Bardet-Biedl syndrome and related syndromic ciliopathies

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3481343

29 September 2017

Selective inhibition of a regulatory subunit of protein phosphatase 1 restores proteostasis

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29 September 2017

Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophy

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29 September 2017

ER-targeted Bcl-2 and inhibition of ER-associated caspase-12 rescue cultured immortalized cells from ethanol toxicity

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29 September 2017

Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78

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29 September 2017

Pharmacological manipulation of gain-of-function and dominant-negative mechanisms in rhodopsin retinitis pigmentosa.

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29 September 2017

Chemical chaperones reduce endoplasmic reticulum stress and prevent mutant HFE aggregate formation

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29 September 2017

Adaptation to ER stress is mediated by differential stabilities of pro-survival and pro-apoptotic mRNAs and proteins

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29 September 2017

Characterization of rhodopsin P23H-induced retinal degeneration in a Xenopus laevis model of retinitis pigmentosa

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29 September 2017

Characterization of the hemin-sensitive eukaryotic initiation factor 2alpha kinase from mouse nonerythroid cells

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29 September 2017

The endoplasmic reticulum stress response factor CHOP-10 protects against hypoxia-induced neuronal death.

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3 June 2018

Exome sequencing identifies mutations in LZTFL1, a BBSome and smoothened trafficking regulator, in a family with Bardet--Biedl syndrome with situs inversus and insertional polydactyly.

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21 January 2018

Activation of endoplasmic reticulum stress in degenerating photoreceptors of the rd1 mouse

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21 January 2018

Photoreceptor vitality in organotypic cultures of mature vertebrate retinas validated by light-dependent molecular movements.

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21 January 2018

Acidosis causes endoplasmic reticulum stress and caspase-12-mediated astrocyte death.

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21 January 2018

Valproic acid-mediated neuroprotection and regeneration in injured retinal ganglion cells.

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https://api.crossref.org/works/10.1074%2FJBC.M112.386821

21 January 2018

Valproate protects the retina from endoplasmic reticulum stress-induced apoptosis after ischemia-reperfusion injury.

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https://api.crossref.org/works/10.1074%2FJBC.M112.386821

21 January 2018

Rapid B cell receptor-induced unfolded protein response in nonsecretory B cells correlates with pro- versus antiapoptotic cell fate.

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21 January 2018

Intraflagellar transport proteins in ciliogenesis of photoreceptor cells

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https://api.crossref.org/works/10.1074%2FJBC.M112.386821

21 January 2018

Therapeutic potential of valproic acid for retinitis pigmentosa

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https://pubmed.ncbi.nlm.nih.gov/22869374

12 December 2020

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The conclusions of Clemson et al concerning valproic acid are premature

1 reference

https://pubmed.ncbi.nlm.nih.gov/22869374

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Lack of scientific rationale for use of valproic acid for retinitis pigmentosa

1 reference

https://pubmed.ncbi.nlm.nih.gov/22869374

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The use of quantitative RT-PCR to measure mRNA expression in a rat model of focal ischemia--caspase-3 as a case study

1 reference

https://pubmed.ncbi.nlm.nih.gov/22869374

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Regulation of internal ribosomal entry site-mediated translation by phosphorylation of the translation initiation factor eIF2alpha

1 reference

https://pubmed.ncbi.nlm.nih.gov/22869374

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ER signaling in unfolded protein response

1 reference

https://pubmed.ncbi.nlm.nih.gov/22869374

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Differential display PCR reveals novel targets for the mood-stabilizing drug valproate including the molecular chaperone GRP78

1 reference

https://pubmed.ncbi.nlm.nih.gov/22869374

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Transcriptional induction of mammalian ER quality control proteins is mediated by single or combined action of ATF6alpha and XBP1

1 reference

https://pubmed.ncbi.nlm.nih.gov/22869374

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1074/JBC.M112.386821

1 reference

31 January 2017

0 references

1 reference

31 January 2017

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