(Q37414030)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19283346%20AND%20SRC:MED&resulttype=core&format=json

23 January 2020

review article

1 reference

title

Multicellular models of Friedreich ataxia (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19283346%20AND%20SRC:MED&resulttype=core&format=json

23 January 2020

1 reference

1 reference

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19283346%20AND%20SRC:MED&resulttype=core&format=json

23 January 2020

author name string

Hélène Puccio

series ordinal

1

1 reference

20 August 2017

publication date

1 March 2009

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19283346%20AND%20SRC:MED&resulttype=core&format=json

23 January 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19283346%20AND%20SRC:MED&resulttype=core&format=json

23 January 2020

volume

256 Suppl 1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19283346%20AND%20SRC:MED&resulttype=core&format=json

23 January 2020

page(s)

18-24

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19283346%20AND%20SRC:MED&resulttype=core&format=json

https://scigraph.springernature.com/pub.10.1007/s00415-009-1004-1

23 January 2020

exact match

0 references

cites work

Ultrastructural observations on spinal ganglion biopsy in Friedreich's ataxia: a preliminary report

1 reference

12 December 2020

The cardiomyopathy of Friedreich's ataxia morphological observations in 3 cases

1 reference

12 December 2020

RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila

1 reference

12 December 2020

Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

1 reference

12 December 2020

Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues

1 reference

12 December 2020

Glucose metabolism alterations in Friedreich's ataxia

1 reference

12 December 2020

HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model

1 reference

12 December 2020

Causative role of oxidative stress in a Drosophila model of Friedreich ataxia.

1 reference

12 December 2020

Iron and iron-responsive proteins in the cardiomyopathy of Friedreich's ataxia

1 reference

12 December 2020

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

1 reference

12 December 2020

Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.

1 reference

12 December 2020

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

1 reference

12 December 2020

GAA repeat instability in Friedreich ataxia YAC transgenic mice.

1 reference

12 December 2020

Breaking the silence in Friedreich's ataxia

1 reference

12 December 2020

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

1 reference

12 December 2020

Drug Insight: antioxidant therapy in inherited ataxias

1 reference

12 December 2020

Frataxin knockin mouse

1 reference

12 December 2020

Relationship between mitochondrial electron transport chain dysfunction, development, and life extension in Caenorhabditis elegans

1 reference

12 December 2020

Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant

1 reference

12 December 2020

A non-essential function for yeast frataxin in iron-sulfur cluster assembly

1 reference

12 December 2020

The mitochondrial permeability transition in cell death: a common mechanism in necrosis, apoptosis and autophagy

1 reference

12 December 2020

Somatic instability of the expanded GAA triplet-repeat sequence in Friedreich ataxia progresses throughout life

1 reference

12 December 2020

Therapeutics development for triplet repeat expansion diseases

1 reference

12 December 2020

Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia

1 reference

12 December 2020

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

1 reference

12 December 2020

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

1 reference

12 December 2020

Lipofuscin: mechanisms of age-related accumulation and influence on cell function

1 reference

12 December 2020

Friedreich ataxia: the oxidative stress paradox

1 reference

12 December 2020

Reduced expression of frataxin extends the lifespan of Caenorhabditis elegans

1 reference

12 December 2020

Friedreich ataxia: a paradigm for mitochondrial diseases

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia

1 reference

12 December 2020

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

1 reference

12 December 2020

Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia

1 reference

12 December 2020

Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation

1 reference

12 December 2020

Impaired respiration is positively correlated with decreased life span in Caenorhabditis elegans models of Friedreich Ataxia

1 reference

12 December 2020

The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins

1 reference

12 December 2020

Friedreich's ataxia with isolated vitamin E deficiency: a neuropathological study of a Tunisian patient

1 reference

12 December 2020

Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm

1 reference

12 December 2020

Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity

1 reference

12 December 2020

10.1007/S00415-009-1004-1

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19283346%20AND%20SRC:MED&resulttype=core&format=json

23 January 2020

1 reference