Associated Papers
Integration of Evolutionary Analysis With RFdiffusion for De Novo Design of Aggregation-Resistant Frataxin.
Kırboğa KK et al.
Proteins, 2026
Nanobodies as tools for studying human frataxin biology.
Pignataro MF et al.
Communications Biology, 2026
An In Silico Analysis of Genetic Variants and Structural Modeling of the Human Frataxin Protein in Friedreich's Ataxia.
Da Conceição LMA et al.
International Journal of Molecular Sciences, 2024
CRISPR/Cas9-based edition of frataxin gene in Dictyostelium discoideum.
Gentili HG et al.
The Biochemical Journal, 2023
In vivo overexpression of frataxin causes toxicity mediated by iron-sulfur cluster deficiency.
Huichalaf C et al.
Molecular Therapy. Methods & Clinical Development, 2022
Protein Mutations and Stability, a Link with Disease: The Case Study of Frataxin.
Puglisi R
Biomedicines, 2022
Conformational stability, dynamics and function of human frataxin: Tryptophan side chain interplay.
Espeche LD et al.
Archives of Biochemistry and Biophysics, 2022
Recent Advances in the Elucidation of Frataxin Biochemical Function Open Novel Perspectives for the Treatment of Friedreich's Ataxia.
Monfort B et al.
Frontiers in Neuroscience, 2022
A Combined Spectroscopic and In Silico Approach to Evaluate the Interaction of Human Frataxin with Mitochondrial Superoxide Dismutase.
Doni D et al.
Biomedicines, 2021
Molecular Details of the Frataxin-Scaffold Interaction during Mitochondrial Fe-S Cluster Assembly.
Campbell CJ et al.
International Journal of Molecular Sciences, 2021
Relationship between activity and stability: Design and characterization of stable variants of human frataxin.
Castro IH et al.
Archives of Biochemistry and Biophysics, 2020
Exploring iron-binding to human frataxin and to selected Friedreich ataxia mutants by means of NMR and EPR spectroscopies.
Bellanda M et al.
Biochimica Et Biophysica Acta. Proteins and Proteomics, 2019
Structure of the human frataxin-bound iron-sulfur cluster assembly complex provides insight into its activation mechanism.
Fox NG et al.
Nature Communications, 2019
Human frataxin activates Fe-S cluster biosynthesis by facilitating sulfur transfer chemistry.
Bridwell-Rabb J et al.
Biochemistry, 2014
The alteration of the C-terminal region of human frataxin distorts its structural dynamics and function.
Faraj SE et al.
The Febs Journal, 2014
Friedreich's ataxia and other hereditary ataxias in Greece: an 18-year perspective.
Koutsis G et al.
Journal of the Neurological Sciences, 2014
Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stress.
Gomes CM et al.
Oxidative Medicine and Cellular Longevity, 2013
Understanding the genetic and molecular pathogenesis of Friedreich's ataxia through animal and cellular models.
Martelli A et al.
Disease Models & Mechanisms, 2012
Structure-function analysis of Friedreich's ataxia mutants reveals determinants of frataxin binding and activation of the Fe-S assembly complex.
Bridwell-Rabb J et al.
Biochemistry, 2011
Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex.
Schmucker S et al.
Plos One, 2011
Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.
Santos R et al.
Antioxidants & Redox Signaling, 2010
Iron-binding activity in yeast frataxin entails a trade off with stability in the alpha1/beta1 acidic ridge region.
Correia AR et al.
The Biochemical Journal, 2010
Frataxin gene point mutations in Italian Friedreich ataxia patients.
Gellera C et al.
Neurogenetics, 2007
Investigation of recessive ataxia loci in patients with young age of onset.
Zühlke C et al.
Neuropediatrics, 2007
Extension of the mutation spectrum in Friedreich's ataxia: detection of an exon deletion and novel missense mutations.
Zühlke CH et al.
European Journal of Human Genetics : Ejhg, 2004