Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia (Q34150158)

31 July 2017

title

Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia (English)

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31 July 2017

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Sara Anjomani Virmouni

1

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31 July 2017

Chiranjeevi Sandi

2

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31 July 2017

Sahar Al-Mahdawi

3

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31 July 2017

Mark A Pook

4

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31 July 2017

language of work or name

English

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publication date

8 September 2014

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31 July 2017

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31 July 2017

volume

9

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31 July 2017

issue

9

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31 July 2017

page(s)

e107416

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Creative Commons Attribution 4.0 International

31 July 2017

copyright license

start time

8 September 2014

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April 2022 Public Data File from Crossref

copyright status

copyrighted

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cites work

Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell models

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Central role and mechanisms of β-cell dysfunction and death in friedreich ataxia-associated diabetes

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Friedreich's ataxia: pathology, pathogenesis, and molecular genetics

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Development of histone deacetylase inhibitors as therapeutics for neurological disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Clinical Use of Interferon-gamma

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Friedreich ataxia: the clinical picture

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Lateral-flow immunoassay for the frataxin protein in Friedreich's ataxia patients and carriers

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

IFN-gamma: recent advances in understanding regulation of expression, biological functions, and clinical applications.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Frataxin deficiency in pancreatic islets causes diabetes due to loss of beta cell mass

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

The molecular basis of Friedreich ataxia.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4157886

GAA repeat instability in Friedreich ataxia YAC transgenic mice.

5 July 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0107416

The GAA triplet-repeat is unstable in the context of the human FXN locus and displays age-dependent expansions in cerebellum and DRG in a transgenic mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0107416

The dentate nucleus in Friedreich's ataxia: the role of iron-responsive proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0107416

Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0107416

Increased iron in the dentate nucleus of patients with Friedrich's ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0107416

Human BAC-mediated rescue of the Friedreich ataxia knockout mutation in transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0107416

21 January 2018

Frataxin knockin mouse

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0107416

Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis

21 January 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/25198290

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/25198290

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1371/JOURNAL.PONE.0107416

1 reference

31 July 2017

0 references

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31 July 2017

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