The first cellular models based on frataxin missense mutations that reproduce spontaneously the defects associated with Friedreich ataxia

PLoS ONE

Volumn 4, Issue 7, 2009, Pages

  Calmels, Nadège ^a,d,e   Schmucker, Stéphane ^a,d,e   Wattenhofer Donzé, Marie ^a,e   Martelli, Alain ^a,e   Vaucamps, Nadège ^a   Reutenauer, Laurence ^a,c   Messaddeq, Nadia ^a,b   Bouton, Cécile ^c   Koenig, Michel ^a,b,c,d,e   Puccio, Hélène ^a,b,c,d,e  

^a INSTITUT DE GÉNÉTIQUE ET DE BIOLOGIE MOLÉCULAIRE ET CELLULAIRE   (France)

^b INSERM   (France)

^c CNRS   (France)

^d UNIVERSITÉ DE STRASBOURG   (France)

^e l'alimentation et l'Environnement   (France)

Author keywords

[No Author keywords available]

Indexed keywords

CRE RECOMBINASE; ENHANCED GREEN FLUORESCENT PROTEIN; FRATAXIN; IRON SULFUR PROTEIN; MITOCHONDRIAL ENZYME; IRON BINDING PROTEIN;

ALLELE; ANIMAL CELL; ARTICLE; CELL DEATH; CONTROLLED STUDY; DISEASE MODEL; DISEASE SEVERITY; ENZYME ACTIVITY; FIBROBLAST; FRIEDREICH ATAXIA; MISSENSE MUTATION; MITOSIS INHIBITION; MOUSE; NONHUMAN; OXIDATIVE STRESS; PHENOTYPE; PROTEIN EXPRESSION; ANIMAL; GENETICS; HETEROZYGOTE; TRANSGENIC MOUSE;

MURINAE;

ANIMALS; FRIEDREICH ATAXIA; HETEROZYGOTE; IRON-BINDING PROTEINS; MICE; MICE, TRANSGENIC; MUTATION, MISSENSE;

EID: 67749148227     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0006379     Document Type: Article

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