Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins

FEBS Journal

Volumn 276, Issue 4, 2009, Pages 1036-1047

  Guillon, Blanche ^a   Bulteau, Anne Laure ^b   Wattenhofer Donzé, Marie ^c,d   Schmucker, Stéphane ^c,e   Friguet, Bertrand ^b   Puccio, Hélène ^a,c,d,e,f   Drapier, Jean Claude ^a   Bouton, Cécile ^a,g  

^a CNRS   (France)

^b UNIVERSITÉ PARIS DESCARTES   (France)

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

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

^e UNIVERSITÉ DE STRASBOURG   (France)

^f INSERM   (France)

^g INSTITUT DE CHIMIE DES SUBSTANCES NATURELLES   (France)

Author keywords

ClpP; Frataxin; Friedreich ataxia; Iron sulfur cluster; Lon protease

Indexed keywords

ADENOSINE TRIPHOSPHATE; CREATINE KINASE MM; ENDOPEPTIDASE CLP; ENDOPEPTIDASE LA; FRATAXIN; IRON SULFUR PROTEIN; MESSENGER RNA; MUTANT PROTEIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CARBONYLATION; CONTROLLED STUDY; DISEASE COURSE; ENZYME ACTIVITY; ENZYME DEGRADATION; ENZYME METABOLISM; FRIEDREICH ATAXIA; GENE EXPRESSION REGULATION; HEART; HEART MITOCHONDRION; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN FOLDING; UPREGULATION;

ADENOSINE TRIPHOSPHATE; ANIMALS; CREATINE KINASE, MM FORM; ENDOPEPTIDASE CLP; FRIEDREICH ATAXIA; IRON-BINDING PROTEINS; IRON-SULFUR PROTEINS; MICE; MICE, TRANSGENIC; MITOCHONDRIAL PROTEINS; MUTATION; MYOCARDIUM; PROTEASE LA; UP-REGULATION;

EID: 58849111392     PISSN: 1742464X     EISSN: 17424658     Source Type: Journal    
DOI: 10.1111/j.1742-4658.2008.06847.x     Document Type: Article

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