E3 Ligase RNF126 Directly Ubiquitinates Frataxin, Promoting Its Degradation: Identification of a Potential Therapeutic Target for Friedreich Ataxia

Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion

Victoria Campuzano, Laura Montermini, María Dolores Moltó et al. · Science · 1996 · 2.7K citations

Ubiquitin-dependent c-Jun degradation in vivo is mediated by the δ domain

Mathias Treier, Lena Staszewski, Dirk Bohmann · Cell · 1994 · 927 citations

Signal Transduction, Cell Regulation, Molecular Regulation +10

Frataxin is Reduced in Friedreich Ataxia Patients and is Associated with Mitochondrial Membranes

Victoria Campuzano, Laura Montermini, Yves Lutz et al. · Human Molecular Genetics · 1997 · 734 citations

Mitophagy, Pathology, Cell Death +16

Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia

David Herman, Kai Jenssen, Ryan Burnett et al. · Nature Chemical Biology · 2006 · 432 citations · Full text

Histone Modifications, Chromatin, Chromatin Remodeling +6

Frataxin Acts as an Iron Chaperone Protein to Modulate Mitochondrial Aconitase Activity

Anne-Laure Bulteau, Heather A. O’Neill, M C Kennedy et al. · Science · 2004 · 368 citations

Reductive Stress, Redox Signaling, Biochemistry +13