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Animal and cellular models of Friedreich ataxia.

author

Helene Puccio

published in: Journal of Neurochemistry

date of publication: 2013-08-01

language: English

main subject: Friedreich ataxia

Cites articles 102

Date

Title

Authors

2012-01-01

Pms2 suppresses large expansions of the (GAA·TTC)n sequence in neuronal tissues

Ricardo Mouro Pinto
2012-02-15

Efficient differentiation of human induced pluripotent stem cells generates cardiac cells that provide protection following myocardial infarction in the rat.
2012-03-22

Neurons derived from human embryonic stem cells extend long-distance axonal projections through growth along host white matter tracts after intra-cerebral transplantation

Clare L Parish, Jordan Wright, Christopher A Reid, Mirella Dottori, Lachlan H Thompson
2012-03-01

Life in the fast lane: mammalian disease models in the genomics era.
2012-07-13

Role of mismatch repair enzymes in GAA·TTC triplet-repeat expansion in Friedreich ataxia induced pluripotent stem cells

Peter Dervan, Elisabetta Soragni
2012-04-01

FXN methylation predicts expression and clinical outcome in Friedreich ataxia

Jeffrey Craig, Simone M Rowley, Nick Wong, Richard Saffery, Martin Delatycki
2012-01-20

The mismatch repair system protects against intergenerational GAA repeat instability in a Friedreich ataxia mouse model
2012-07-11

DNA mismatch repair complex MutSβ promotes GAA·TTC repeat expansion in human cells
2012-01-30

Is Friedreich ataxia an epigenetic disorder?
2012-05-29

Robust cardiomyocyte differentiation from human pluripotent stem cells via temporal modulation of canonical Wnt signaling

Xiaojun Lian, Kexian Zhu, Timothy J. Kamp
2012-06-26

Clinical data and characterization of the liver conditional mouse model exclude neoplasia as a non-neurological manifestation associated with Friedreich's ataxia

Kathrin Fedosov, Jörg B. Schulz, Helene Puccio, Susan Perlman
2012-03-01

Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease
2012-03-06

Friedreich's ataxia reveals a mechanism for coordinate regulation of oxidative metabolism via feedback inhibition of the SIRT3 deacetylase
2011-10-11

Friedreich's ataxia: the vicious circle hypothesis revisited

Aurélien Bayot, Renata Santos, Jean-Michel Camadro
2011-05-01

Human ESC-derived neural crest model reveals a key role for SOX2 in sensory neurogenesis

Flavio Cimadamore, Marianne Bronner, Alexey V Terskikh
2011-06-07

Differential expression of PGC-1α and metabolic sensors suggest age-dependent induction of mitochondrial biogenesis in Friedreich ataxia fibroblasts

José Luis García-Giménez, Francisco Dasí, Francesc Palau
2011-02-04

Stage-specific optimization of activin/nodal and BMP signaling promotes cardiac differentiation of mouse and human pluripotent stem cell lines
2011-07-10

Hyperexpansion of GAA repeats affects post-initiation steps of FXN transcription in Friedreich's ataxia

Sharon Dent
2011-04-01

Friedreich's ataxia: pathology, pathogenesis, and molecular genetics
2011-09-01

Generation of induced pluripotent stem cell lines from Friedreich ataxia patients

Jun Liu, Duncan E Crombie, Mirella Dottori, Alice Pébay, Martin Delatycki
2011-03-01

Cardiomyopathy of Friedreich's ataxia: use of mouse models to understand human disease and guide therapeutic development
2011-06

Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model
2011-01-26

Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex

Marie Wattenhofer, Helene Puccio
2011-06-08

Mortality in Friedreich ataxia

Katherine Mathews
2011-11-23

A TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model
2011-05-24

Several rAAV vectors efficiently cross the blood-brain barrier and transduce neurons and astrocytes in the neonatal mouse central nervous system
2010-07-08

A rapid, noninvasive immunoassay for frataxin: utility in assessment of Friedreich ataxia

Susan Perlman
2010-11-01

Friedreich's ataxia induced pluripotent stem cells model intergenerational GAA⋅TTC triplet repeat instability

Joel M. Gottesfeld, Louise C Laurent, Jeanne Loring
2010-12-02

Repeat expansion affects both transcription initiation and elongation in friedreich ataxia cells
2010-03-18

Derivation of neural crest cells from human pluripotent stem cells.

Lorenz Studer
2010-03-01

Repeat instability as the basis for human diseases and as a potential target for therapy
2010-04-07

PGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia

Fabio Acquaviva

Article - wd:Q38121944

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